Name Impact
?

 Papers Institution Country Funding

Cleveland, Don W

 555.6 14 University of California at San Diego USA $13,609,881.00

Gitler, Aaron D

 483.4 15 Stanford University School of Medicine USA $5,038,407.00

Trojanowski, John Q

 462.9 21 University of Pennsylvania School of Medicine USA $34,223,307.00

Shaw, Christopher E

 366.8 10 King's College London UK -

Lee, Virginia M-Y

 347.7 13 University of Pennsylvania School of Medicine USA $951,941.00

Van Broeckhoven, C

 302.9 13 University of Antwerp-CDE Belgium -

Mackenzie, Ian R A

 290.3 11 University Hospital of Zürich Switzerland -

Mann, David M A

 278.3 12 University of Manchester UK -

Haass, Christian

 240.9 10 Ludwig-Maximilians-University and German Center for Neurodegenerative Diseases (DZNE) Munich Germany -

Petrucelli, Leonard

 239.4 14 Mayo Clinic USA $4,708,353.00

Baralle, Francisco E

 232.5 12 International Centre for Genetic Engineering and Biotechnology (ICGEB), 34012 Trieste Italy -

Dickson, Dennis W

 229.6 10 Mayo Clinic USA -

Neumann, Manuela

 212.8 7 University of British Columbia and Vancouver General Hospital Canada -

Wakabayashi, Koichi

 208.1 22 Hirosaki University Graduate School of Medicine Japan -

Landers, J E

 199.7 8 University of Massachusetts Medical School USA -

Kawakami, Hideshi

 196.8 5 Hiroshima University Japan -

Rademakers, Rosa

 189.8 13 Mayo Clinic USA $3,824,782.00

Shaw, Pamela J

 186.9 13 University of Sheffield UK -

Takahashi, Hitoshi

 186.8 11 Nishi-Niigata Chuo National Hospital Japan -

Shen, Che-Kun James

 176.1 7 Institute of Life Science Taiwan -

Traynor, Bryan J

 173.8 6 National Institute on Aging USA -

Edbauer, Dieter

 169.5 5 German Center for Neurodegenerative Diseases (DZNE), Munich Germany -

Rouleau, G A

 168 13 Notre-Dame Hospital Canada -

Robberecht, Wim

 161 7 VIB Vesalius Research Center, Leuven Belgium -

van Den Berg, Leonard H

 159.7 16 University Medical Center Utrecht Netherlands -

Siddique, T

 151.3 6 Northwestern University Feinberg School of Medicine USA -

Xia, Xu Gang

 150 6 Thomas Jefferson University USA -

Bosco, D A

 149.3 3 Massachusetts General Hospital USA -

Wszolek, Zbigniew K

 145.3 5 Mayo Clinic USA $1,372,564.00

Cairns, Nigel J

 144.2 10 Aston University UK -

Julien, Jean-Pierre

 135.8 9 Department of Psychiatry and Neuroscience of Laval University Canada -

Baloh, Robert H

 132.3 9 Washington University School of Medicine USA -

Kwak, Shin

 129.5 11 The University of Tokyo Japan -

Parker, J Alex

 127 8 CRCHUM, Université de Montréal, Montréal, Québec Canada -

Akiyama, Haruhiko

 124.2 6 Tokyo Institute of Psychiatry Japan -

Grossman, Murray

 123.1 10 University of Pennsylvania School of Medicine USA $9,846,530.00

Blair, I P

 119.4 8 Concord Hospital NSW 2139 Australia -

Bonini, Nancy M

 110.7 2 University of Pennsylvania USA $4,022,711.00

Suzuki, Norihiro

 106.2 4 Keio University Japan -

Josephs, Keith A

 99.6 10 Mayo Clinic Rochester USA $2,578,753.00

Sobue, Gen

 95 12 Nagoya University Graduate School of Medicine Japan -

Strong, Michael J

 94.4 9 Robarts Research Institute Canada -

Drapeau, Pierre

 93.2 4 Department of Pathology and Cell Biology and Groupe de Recherche sur le Système Nerveux Central, Université de Montréal, Montréal, QC, … Canada -

Tibbetts, Randal S

 92.5 6 University of Wisconsin School of Medicine and Public Health USA -

Hardiman, Orla

 89.7 5 Trinity College Dublin Ireland -

Klein, Ronald L

 86.8 5 Louisiana State University Health Sciences Center USA $190,762.00

Revesz, Tamas

 86 6 University College London UK -

Oddo, Salvatore

 86 5 University of Texas Health Science Center at San Antonio USA $1,690,118.00

Kumar-Singh, Samir

 85.3 5 Neurodegenerative Brain Diseases Group, Department of Molecular Genetics, VIB, Universiteitsplein 1, 2610, Antwerp Belgium -

Isaacs, Adrian M

 82.5 4 UCL Institute of Neurology UK -
Name Institution Date Impact Publication

Landers, John E

 University of Massachusetts Medical School 08/23/2012 36.28 Nature
Mutations in the profilin 1 gene cause familial amyotrophic lateral sclerosis. Abstract

Siddique, Teepu

 Northwestern University 08/21/2011 36.28 Nature
Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia. Abstract

Gitler, Aaron D

 Stanford University School of Medicine 08/26/2010 36.28 Nature
Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS. Abstract

Kawakami, Hideshi

 Hiroshima University 04/28/2010 36.28 Nature
Mutations of optineurin in amyotrophic lateral sclerosis. Abstract

Bonini, Nancy M

 University of Pennsylvania 12/15/2013 35.53 Nature genetics
Therapeutic modulation of eIF2α phosphorylation rescues TDP-43 toxicity in amyotrophic lateral sclerosis disease models. Abstract

Cleveland, Don W

 University of California at San Diego 11/29/2012 35.53 Nature genetics
TDP-43 toxicity and the usefulness of junk. Abstract

Gitler, Aaron D

 Stanford University School of Medicine 10/28/2012 35.53 Nature genetics
Inhibition of RNA lariat debranching enzyme suppresses TDP-43 toxicity in ALS disease models. Abstract

Lee, Virginia M-Y

 University of Pennsylvania School of Medicine 02/14/2010 35.53 Nature genetics
Common variants at 7p21 are associated with frontotemporal lobar degeneration with TDP-43 inclusions. Abstract

Wszolek, Zbigniew K

 Mayo Clinic 01/11/2009 35.53 Nature genetics
DCTN1 mutations in Perry syndrome. Abstract

Lee, Virginia M Y

 University of Pennsylvania School of Medicine 07/05/2013 32.40 Cell
Distinct α-synuclein strains differentially promote tau inclusions in neurons. Abstract

Cleveland, Don W

 University of California at San Diego 10/31/2011 32.40 Cell
The seeds of neurodegeneration: prion-like spreading in ALS. Abstract

Cleveland, Don W

 University of California at San Diego 03/23/2009 32.40 Cell
Rethinking ALS: the FUS about TDP-43. Abstract

Edbauer, Dieter

 German Center for Neurodegenerative Diseases (DZNE), Munich 02/07/2013 31.20 Science (New York, N.Y.)
The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD/ALS. Abstract

Bosco, D A

 Massachusetts General Hospital 03/02/2009 31.20 Science (New York, N.Y.)
Mutations in the FUS/TLS gene on chromosome 16 cause familial amyotrophic lateral sclerosis. Abstract

Shaw, Christopher E

 King's College London 03/02/2009 31.20 Science (New York, N.Y.)
Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6. Abstract

Trojanowski, John Q

 University of Pennsylvania School of Medicine 11/30/2011 30.44 Nature reviews. Neuroscience
Gains or losses: molecular mechanisms of TDP43-mediated neurodegeneration. Abstract

Van Broeckhoven, Christine

 Neurodegenerative Brain Diseases Group, Department of Molecular Genetics, VIB, Universiteitsplein 1, B-2610 Antwerpen 06/24/2010 25.73 Annual review of neuroscience
Molecular pathways of frontotemporal lobar degeneration. Abstract

Traynor, Bryan J

 National Institutes of Health 03/09/2012 23.46 The Lancet. Neurology
Frequency of the C9orf72 hexanucleotide repeat expansion in patients with amyotrophic lateral sclerosis and frontotemporal dementia: a cross-sectional study. Abstract

Hardiman, Orla

 Trinity College Dublin 02/03/2012 23.46 The Lancet. Neurology
Cognitive and clinical characteristics of patients with amyotrophic lateral sclerosis carrying a C9orf72 repeat expansion: a population-based cohort study. Abstract

Van Broeckhoven, Christine

 Neurodegenerative Brain Diseases Group, Department of Molecular Genetics, VIB, Universiteitsplein 1, B-2610 Antwerpen 12/07/2011 23.46 The Lancet. Neurology
A C9orf72 promoter repeat expansion in a Flanders-Belgian cohort with disorders of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum: a gene identification study. Abstract

Robberecht, Wim

 VIB Vesalius Research Center, Leuven 02/25/2011 23.46 The Lancet. Neurology
Neuroinflammation in amyotrophic lateral sclerosis: role of glial activation in motor neuron disease. Abstract

Neumann, Manuela

 University of British Columbia and Vancouver General Hospital 09/24/2010 23.46 The Lancet. Neurology
TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia. Abstract

Robberecht, Wim

 VIB Vesalius Research Center, Leuven 11/05/2012 22.46 Nature medicine
EPHA4 is a disease modifier of amyotrophic lateral sclerosis in animal models and in humans. Abstract

Traynor, Bryan J

 National Institute on Aging 12/26/2013 15.53 Nature neuroscience
State of play in amyotrophic lateral sclerosis genetics. Abstract

Gitler, Aaron D

 Stanford University School of Medicine 05/26/2013 15.53 Nature neuroscience
Exome sequencing to identify de novo mutations in sporadic ALS trios. Abstract

Cleveland, Don W

 University of California at San Diego 02/27/2011 15.53 Nature neuroscience
Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43. Abstract

Cleveland, Don W

 University of California at San Diego 08/12/2013 14.73 Neuron
Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis. Abstract

Petrucelli, Leonard

 Mayo Clinic 02/12/2013 14.73 Neuron
Unconventional translation of C9ORF72 GGGGCC expansion generates insoluble polypeptides specific to c9FTD/ALS. Abstract

Rademakers, Rosa

 Mayo Clinic 09/21/2011 14.73 Neuron
Expanded GGGGCC hexanucleotide repeat in noncoding region of C9ORF72 causes chromosome 9p-linked FTD and ALS. Abstract

Traynor, Bryan J

 National Institute on Aging 09/21/2011 14.73 Neuron
A hexanucleotide repeat expansion in C9ORF72 is the cause of chromosome 9p21-linked ALS-FTD. Abstract

Shaw, Christopher E

 King's College London 12/14/2010 14.73 Neuron
Capturing VCP: another molecular piece in the ALS jigsaw puzzle. Abstract

Traynor, Bryan J

 National Institute on Aging 12/14/2010 14.73 Neuron
Exome sequencing reveals VCP mutations as a cause of familial ALS. Abstract

Cleveland, Don W

 University of California at San Diego 08/27/2010 14.73 Neuron
Misfolded mutant SOD1 directly inhibits VDAC1 conductance in a mouse model of inherited ALS. Abstract

Van Broeckhoven, Christine

 Neurodegenerative Brain Diseases Group, Department of Molecular Genetics, VIB, Universiteitsplein 1, B-2610 Antwerpen 06/07/2013 14.23 Trends in neurosciences
Current insights into the C9orf72 repeat expansion diseases of the FTLD/ALS spectrum. Abstract

Haass, Christian

 Ludwig-Maximilians-University and German Center for Neurodegenerative Diseases (DZNE) Munich 06/22/2011 14.23 Trends in neurosciences
TDP-43 and FUS: a nuclear affair. Abstract

Julien, Jean-Pierre

 Department of Psychiatry and Neuroscience of Laval University 11/14/2011 13.85 The Journal of experimental medicine
Deregulation of TDP-43 in amyotrophic lateral sclerosis triggers nuclear factor κB-mediated pathogenic pathways. Abstract

Shen, Che-Kun James

 Institute of Life Science 07/26/2010 13.85 The Journal of experimental medicine
Elevated expression of TDP-43 in the forebrain of mice is sufficient to cause neurological and pathological phenotypes mimicking FTLD-U. Abstract

Cleveland, Don W

 University of California at San Diego 05/07/2012 13.66 Cell metabolism
Elevated PGC-1α activity sustains mitochondrial biogenesis and muscle function without extending survival in a mouse model of inherited ALS. Abstract

Xia, Xu-Gang

 Thomas Jefferson University 12/12/2011 13.06 The Journal of clinical investigation
Mutant TDP-43 in motor neurons promotes the onset and progression of ALS in rats. Abstract

Lee, Virginia M-Y

 University of Pennsylvania School of Medicine 01/04/2011 13.06 The Journal of clinical investigation
Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice. Abstract

Mackenzie, Ian R

 University of Toronto 06/26/2012 12.46 Nature reviews. Neurology
Advances in understanding the molecular basis of frontotemporal dementia. Abstract

Josephs, Keith A

 Mayo Clinic Rochester 01/31/2012 12.46 Nature reviews. Neurology
Neuroimaging in frontotemporal lobar degeneration--predicting molecular pathology. Abstract

Shaw, Pamela J

 University of Sheffield 11/04/2011 12.46 Nature reviews. Neurology
Molecular pathways of motor neuron injury in amyotrophic lateral sclerosis. Abstract

Trojanowski, John Q

 University of Pennsylvania School of Medicine 03/16/2010 12.46 Nature reviews. Neurology
TAR DNA-binding protein 43 in neurodegenerative disease. Abstract

Grossman, Murray

 University of Pennsylvania 02/08/2010 12.46 Nature reviews. Neurology
Primary progressive aphasia: clinicopathological correlations. Abstract

Baralle, Francisco E

 International Centre for Genetic Engineering and Biotechnology (ICGEB), 34012 Trieste 08/02/2012 11.65 Genes & development
Autoregulation of TDP-43 mRNA levels involves interplay between transcription, splicing, and alternative polyA site selection. Abstract

Gitler, Aaron D

 Stanford University School of Medicine 04/26/2011 11.45 PLoS biology
Molecular determinants and genetic modifiers of aggregation and toxicity for the ALS disease protein FUS/TLS. Abstract

Hardiman, Orla

 Trinity College Dublin 09/10/2013 11.08 Annals of neurology
Aggregation of neurologic and neuropsychiatric disease in amyotrophic lateral sclerosis kindreds: a population-based case-control cohort study of familial and sporadic amyotrophic lateral sclerosis. Abstract

Trojanowski, John Q

 University of Pennsylvania School of Medicine 06/19/2013 11.08 Annals of neurology
Stages of pTDP-43 pathology in amyotrophic lateral sclerosis. Abstract

van den Berg, Leonard H

 University Medical Center Utrecht 12/22/2011 11.08 Annals of neurology
Angiogenin variants in Parkinson disease and amyotrophic lateral sclerosis. Abstract

Suzuki, Norihiro

 Keio University 12/08/2010 11.08 Annals of neurology
Nuclear transport impairment of amyotrophic lateral sclerosis-linked mutations in FUS/TLS. Abstract

Siddique, Teepu

 Northwestern University 06/02/2010 11.08 Annals of neurology
FUS-immunoreactive inclusions are a common feature in sporadic and non-SOD1 familial amyotrophic lateral sclerosis. Abstract

Baralle, Francisco E

 International Centre for Genetic Engineering and Biotechnology (ICGEB), 34012 Trieste 04/24/2012 10.84 Trends in biochemical sciences
TDP-43: gumming up neurons through protein-protein and protein-RNA interactions. Abstract

Rouleau, Guy A

 Centre of Excellence in Neurosciences of Université de Montréal, CHUM Research Center, Montreal, Québec 08/02/2012 10.60 American journal of human genetics
Exome sequencing identifies FUS mutations as a cause of essential tremor. Abstract

Trojanowski, John Q

 University of Pennsylvania School of Medicine 07/23/2011 10.35 Trends in molecular medicine
TDP-43 functions and pathogenic mechanisms implicated in TDP-43 proteinopathies. Abstract

Kwak, Shin

 The University of Tokyo 09/24/2013 10.33 EMBO molecular medicine
Rescue of amyotrophic lateral sclerosis phenotype in a mouse model by intravenous AAV9-ADAR2 delivery to motor neurons. Abstract

Gitler, Aaron D

 Stanford University School of Medicine 04/30/2013 10.26 The Journal of cell biology
Stress granules as crucibles of ALS pathogenesis. Abstract

Cleveland, Don W

 University of California at San Diego 10/29/2013 9.81 Proceedings of the National Academy of Sciences of the United States of America
C1q induction and global complement pathway activation do not contribute to ALS toxicity in mutant SOD1 mice. Abstract

Haass, Christian

 Ludwig-Maximilians-University and German Center for Neurodegenerative Diseases (DZNE) Munich 03/01/2013 9.81 Proceedings of the National Academy of Sciences of the United States of America
Loss of ALS-associated TDP-43 in zebrafish causes muscle degeneration, vascular dysfunction, and reduced motor neuron axon outgrowth. Abstract

Cleveland, Don W

 University of California at San Diego 02/04/2013 9.81 Proceedings of the National Academy of Sciences of the United States of America
ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43. Abstract

Petrucelli, Leonard

 Mayo Clinic 12/10/2012 9.81 Proceedings of the National Academy of Sciences of the United States of America
Misregulation of human sortilin splicing leads to the generation of a nonfunctional progranulin receptor. Abstract

Shen, Che-Kun James

 Institute of Life Science 08/29/2012 9.81 Proceedings of the National Academy of Sciences of the United States of America
Autophagy activators rescue and alleviate pathogenesis of a mouse model with proteinopathies of the TAR DNA-binding protein 43. Abstract

Gitler, Aaron D

 Stanford University School of Medicine 11/07/2011 9.81 Proceedings of the National Academy of Sciences of the United States of America
A yeast functional screen predicts new candidate ALS disease genes. Abstract

Cleveland, Don W

 University of California at San Diego 07/12/2010 9.81 Proceedings of the National Academy of Sciences of the United States of America
ALS-associated mutations in TDP-43 increase its stability and promote TDP-43 complexes with FUS/TLS. Abstract

Kumar-Singh, Samir

 Neurodegenerative Brain Diseases Group, Department of Molecular Genetics, VIB, Universiteitsplein 1, 2610, Antwerp 02/03/2010 9.81 Proceedings of the National Academy of Sciences of the United States of America
TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration. Abstract

Baloh, Robert H

 Washington University School of Medicine 10/15/2009 9.81 Proceedings of the National Academy of Sciences of the United States of America
TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration. Abstract

Petrucelli, Leonard

 Mayo Clinic 04/21/2009 9.81 Proceedings of the National Academy of Sciences of the United States of America
Aberrant cleavage of TDP-43 enhances aggregation and cellular toxicity. Abstract

Cleveland, Don W

 University of California at San Diego 02/27/2009 9.81 Proceedings of the National Academy of Sciences of the United States of America
Schwann cells expressing dismutase active mutant SOD1 unexpectedly slow disease progression in ALS mice. Abstract

Sobue, Gen

 Nagoya University Graduate School of Medicine 02/28/2013 9.46 Brain : a journal of neurology
Loss of TDP-43 causes age-dependent progressive motor neuron degeneration. Abstract

Shaw, Pamela J

 University of Sheffield 02/27/2012 9.46 Brain : a journal of neurology
Clinico-pathological features in amyotrophic lateral sclerosis with expansions in C9ORF72. Abstract

Isaacs, Adrian M

 UCL Institute of Neurology 02/27/2012 9.46 Brain : a journal of neurology
Progressive neuronal inclusion formation and axonal degeneration in CHMP2B mutant transgenic mice. Abstract

Mackenzie, Ian R A

 University Hospital of Zürich 02/17/2012 9.46 Brain : a journal of neurology
Clinical and pathological features of familial frontotemporal dementia caused by C9ORF72 mutation on chromosome 9p. Abstract

Trojanowski, John Q

 University of Pennsylvania School of Medicine 11/26/2011 9.46 Brain : a journal of neurology
Neocortical and hippocampal amyloid-β and tau measures associate with dementia in the oldest-old. Abstract

Mackenzie, Ian R A

 University Hospital of Zürich 08/19/2011 9.46 Brain : a journal of neurology
FET proteins TAF15 and EWS are selective markers that distinguish FTLD with FUS pathology from amyotrophic lateral sclerosis with FUS mutations. Abstract

Julien, Jean-Pierre

 Department of Psychiatry and Neuroscience of Laval University 07/13/2011 9.46 Brain : a journal of neurology
Pathological hallmarks of amyotrophic lateral sclerosis/frontotemporal lobar degeneration in transgenic mice produced with TDP-43 genomic fragments. Abstract

Revesz, Tamas

 University College London 07/12/2011 9.46 Brain : a journal of neurology
A comparative clinical, pathological, biochemical and genetic study of fused in sarcoma proteinopathies. Abstract

Mackenzie, Ian R A

 University Hospital of Zürich 08/11/2009 9.46 Brain : a journal of neurology
A new subtype of frontotemporal lobar degeneration with FUS pathology. Abstract

Rademakers, Rosa

 Mayo Clinic 01/21/2009 9.46 Brain : a journal of neurology
Plasma progranulin levels predict progranulin mutation status in frontotemporal dementia patients and asymptomatic family members. Abstract

Neumann, Manuela

 University of British Columbia and Vancouver General Hospital 12/20/2013 9.32 Acta neuropathologica
The neuropathology associated with repeat expansions in the C9ORF72 gene. Abstract

Dickson, Dennis W

 Mayo Clinic 11/16/2013 9.32 Acta neuropathologica
Staging TDP-43 pathology in Alzheimer's disease. Abstract

Isaacs, Adrian M

 UCL Institute of Neurology 10/30/2013 9.32 Acta neuropathologica
C9orf72 frontotemporal lobar degeneration is characterised by frequent neuronal sense and antisense RNA foci. Abstract

Edbauer, Dieter

 German Center for Neurodegenerative Diseases (DZNE), Munich 10/17/2013 9.32 Acta neuropathologica
Bidirectional transcripts of the expanded C9orf72 hexanucleotide repeat are translated into aggregating dipeptide repeat proteins. Abstract

Neumann, Manuela

 University of British Columbia and Vancouver General Hospital 10/06/2013 9.32 Acta neuropathologica
Dipeptide repeat protein pathology in C9ORF72 mutation cases: clinico-pathological correlations. Abstract

Dickson, Dennis W

 Mayo Clinic 08/07/2013 9.32 Acta neuropathologica
Progressive amnestic dementia, hippocampal sclerosis, and mutation in C9ORF72. Abstract

Takahashi, Hitoshi

 Nishi-Niigata Chuo National Hospital 06/28/2013 9.32 Acta neuropathologica
Sporadic ALS with compound heterozygous mutations in the SQSTM1 gene. Abstract

Haass, Christian

 Ludwig-Maximilians-University and German Center for Neurodegenerative Diseases (DZNE) Munich 02/05/2013 9.32 Acta neuropathologica
hnRNP A3 binds to GGGGCC repeats and is a constituent of p62-positive/TDP43-negative inclusions in the hippocampus of patients with C9orf72 mutations. Abstract

Dickson, Dennis W

 Mayo Clinic 11/04/2012 9.32 Acta neuropathologica
Sequence variants in eukaryotic translation initiation factor 4-gamma (eIF4G1) are associated with Lewy body dementia. Abstract

Trojanowski, John Q

 University of Pennsylvania School of Medicine 11/03/2012 9.32 Acta neuropathologica
TDP-43 skeins show properties of amyloid in a subset of ALS cases. Abstract

Dickson, Dennis W

 Mayo Clinic 09/28/2012 9.32 Acta neuropathologica
Tau pathology in frontotemporal lobar degeneration with C9ORF72 hexanucleotide repeat expansion. Abstract

Shaw, Christopher E

 King's College London 09/28/2012 9.32 Acta neuropathologica
Mixed tau, TDP-43 and p62 pathology in FTLD associated with a C9ORF72 repeat expansion and p.Ala239Thr MAPT (tau) variant. Abstract

Trojanowski, John Q

 University of Pennsylvania School of Medicine 09/18/2012 9.32 Acta neuropathologica
TDP-43 pathology in a case of hereditary spastic paraplegia with a NIPA1/SPG6 mutation. Abstract

Shaw, Christopher E

 King's College London 09/09/2012 9.32 Acta neuropathologica
Overexpression of human wild-type FUS causes progressive motor neuron degeneration in an age- and dose-dependent fashion. Abstract

Strong, Michael J

 Robarts Research Institute 09/01/2012 9.32 Acta neuropathologica
Co-aggregation of RNA binding proteins in ALS spinal motor neurons: evidence of a common pathogenic mechanism. Abstract

Mann, David M A

 University of Manchester 08/10/2012 9.32 Acta neuropathologica
Mechanisms of disease in frontotemporal lobar degeneration: gain of function versus loss of function effects. Abstract

van den Berg, Leonard H

 University Medical Center Utrecht 08/02/2012 9.32 Acta neuropathologica
The genetics and neuropathology of amyotrophic lateral sclerosis. Abstract

Mackenzie, Ian R A

 University Hospital of Zürich 07/28/2012 9.32 Acta neuropathologica
Transportin 1 accumulates specifically with FET proteins but no other transportin cargos in FTLD-FUS and is absent in FUS inclusions in ALS with FUS mutations. Abstract

Shen, Che-Kun James

 Institute of Life Science 07/04/2012 9.32 Acta neuropathologica
TDP-43 regulates the mammalian spinogenesis through translational repression of Rac1. Abstract

Trojanowski, John Q

 University of Pennsylvania School of Medicine 04/22/2012 9.32 Acta neuropathologica
CSF biomarkers cutoffs: the importance of coincident neuropathological diseases. Abstract

Gitler, Aaron D

 Stanford University School of Medicine 04/21/2012 9.32 Acta neuropathologica
Distinct TDP-43 pathology in ALS patients with ataxin 2 intermediate-length polyQ expansions. Abstract

Akiyama, Haruhiko

 Tokyo Institute of Psychiatry 04/18/2012 9.32 Acta neuropathologica
Localization of fused in sarcoma (FUS) protein to the post-synaptic density in the brain. Abstract

Wakabayashi, Koichi

 Hirosaki University Graduate School of Medicine 04/07/2012 9.32 Acta neuropathologica
Autophagic adapter protein NBR1 is localized in Lewy bodies and glial cytoplasmic inclusions and is involved in aggregate formation in α-synucleinopathy. Abstract

Trojanowski, John Q

 University of Pennsylvania School of Medicine 03/18/2012 9.32 Acta neuropathologica
Pattern of ubiquilin pathology in ALS and FTLD indicates presence of C9ORF72 hexanucleotide expansion. Abstract

Mackenzie, Ian R A

 University Hospital of Zürich 01/07/2012 9.32 Acta neuropathologica
Clinical and pathological features of amyotrophic lateral sclerosis caused by mutation in the C9ORF72 gene on chromosome 9p. Abstract

Trojanowski, John Q

 University of Pennsylvania School of Medicine 01/01/2012 9.32 Acta neuropathologica
Microglial activation and TDP-43 pathology correlate with executive dysfunction in amyotrophic lateral sclerosis. Abstract

Shaw, Pamela J

 University of Sheffield 11/22/2011 9.32 Acta neuropathologica
Molecular pathology and genetic advances in amyotrophic lateral sclerosis: an emerging molecular pathway and the significance of glial pathology. Abstract

Shaw, Christopher E

 King's College London 11/19/2011 9.32 Acta neuropathologica
p62 positive, TDP-43 negative, neuronal cytoplasmic and intranuclear inclusions in the cerebellum and hippocampus define the pathology of C9orf72-linked FTLD and MND/ALS. Abstract

Dickson, Dennis W

 Mayo Clinic 11/15/2011 9.32 Acta neuropathologica
Clinical and neuropathologic heterogeneity of c9FTD/ALS associated with hexanucleotide repeat expansion in C9ORF72. Abstract

Mann, David M A

 University of Manchester 10/04/2011 9.32 Acta neuropathologica
TDP-43 pathological changes in early onset familial and sporadic Alzheimer's disease, late onset Alzheimer's disease and Down's syndrome: association with age, hippocampal sclerosis and clinical phenotype. Abstract

Trojanowski, John Q

 University of Pennsylvania School of Medicine 09/30/2011 9.32 Acta neuropathologica
Simulated brain biopsy for diagnosing neurodegeneration using autopsy-confirmed cases. Abstract

Revesz, Tamas

 University College London 08/17/2011 9.32 Acta neuropathologica
Transportin1: a marker of FTLD-FUS. Abstract

Revesz, Tamas

 University College London 07/21/2011 9.32 Acta neuropathologica
Globular glial tauopathies (GGT) presenting with motor neuron disease or frontotemporal dementia: an emerging group of 4-repeat tauopathies. Abstract

Kawakami, Hideshi

 Hiroshima University 06/05/2011 9.32 Acta neuropathologica
Clinicopathologic study on an ALS family with a heterozygous E478G optineurin mutation. Abstract

Dickson, Dennis W

 Mayo Clinic 05/26/2011 9.32 Acta neuropathologica
Neuropathological background of phenotypical variability in frontotemporal dementia. Abstract

Neumann, Manuela

 University of British Columbia and Vancouver General Hospital 05/21/2011 9.32 Acta neuropathologica
Pathological heterogeneity in amyotrophic lateral sclerosis with FUS mutations: two distinct patterns correlating with disease severity and mutation. Abstract

Mann, David M A

 University of Manchester 03/20/2011 9.32 Acta neuropathologica
The most common type of FTLD-FUS (aFTLD-U) is associated with a distinct clinical form of frontotemporal dementia but is not related to mutations in the FUS gene. Abstract

Shaw, Christopher E

 King's College London 03/01/2011 9.32 Acta neuropathologica
Optineurin inclusions occur in a minority of TDP-43 positive ALS and FTLD-TDP cases and are rarely observed in other neurodegenerative disorders. Abstract

Mann, David M A

 University of Manchester 01/18/2011 9.32 Acta neuropathologica
Granular expression of prolyl-peptidyl isomerase PIN1 is a constant and specific feature of Alzheimer's disease pathology and is independent of tau, Aβ and TDP-43 pathology. Abstract

Trojanowski, John Q

 University of Pennsylvania School of Medicine 01/12/2011 9.32 Acta neuropathologica
Motor neuron disease clinically limited to the lower motor neuron is a diffuse TDP-43 proteinopathy. Abstract

Neumann, Manuela

 University of British Columbia and Vancouver General Hospital 10/30/2010 9.32 Acta neuropathologica
Distinct pathological subtypes of FTLD-FUS. Abstract

Mann, David M A

 University of Manchester 10/27/2010 9.32 Acta neuropathologica
Pathological correlates of frontotemporal lobar degeneration in the elderly. Abstract

Cairns, Nigel J

 Aston University 10/01/2010 9.32 Acta neuropathologica
The spectrum and severity of FUS-immunoreactive inclusions in the frontal and temporal lobes of ten cases of neuronal intermediate filament inclusion disease. Abstract

Mann, David M A

 University of Manchester 07/29/2010 9.32 Acta neuropathologica
Effect of topographical distribution of α-synuclein pathology on TDP-43 accumulation in Lewy body disease. Abstract

Takahashi, Hitoshi

 Nishi-Niigata Chuo National Hospital 06/09/2010 9.32 Acta neuropathologica
Involvement of the peripheral nervous system in synucleinopathies, tauopathies and other neurodegenerative proteinopathies of the brain. Abstract

Mann, David M A

 University of Manchester 05/30/2010 9.32 Acta neuropathologica
Phosphorylated TDP-43 pathology and hippocampal sclerosis in progressive supranuclear palsy. Abstract

Takahashi, Hitoshi

 Nishi-Niigata Chuo National Hospital 05/26/2010 9.32 Acta neuropathologica
Involvement of Onuf's nucleus in Machado-Joseph disease: a morphometric and immunohistochemical study. Abstract

Isaacs, Adrian M

 UCL Institute of Neurology 05/20/2010 9.32 Acta neuropathologica
FUS pathology defines the majority of tau- and TDP-43-negative frontotemporal lobar degeneration. Abstract

Kwak, Shin

 The University of Tokyo 04/07/2010 9.32 Acta neuropathologica
TDP-43 pathology in sporadic ALS occurs in motor neurons lacking the RNA editing enzyme ADAR2. Abstract

Takahashi, Hitoshi

 Nishi-Niigata Chuo National Hospital 02/07/2010 9.32 Acta neuropathologica
Sporadic four-repeat tauopathy with frontotemporal lobar degeneration, Parkinsonism, and motor neuron disease: a distinct clinicopathological and biochemical disease entity. Abstract

Mackenzie, Ian R

 University of Toronto 10/15/2009 9.32 Acta neuropathologica
FUS pathology in basophilic inclusion body disease. Abstract

Mann, David M A

 University of Manchester 10/13/2009 9.32 Acta neuropathologica
Plasma phosphorylated-TDP-43 protein levels correlate with brain pathology in frontotemporal lobar degeneration. Abstract

Mackenzie, Ian R A

 University Hospital of Zürich 08/09/2009 9.32 Acta neuropathologica
Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion disease. Abstract

Lee, Virginia M-Y

 University of Pennsylvania School of Medicine 08/02/2009 9.32 Acta neuropathologica
Brain progranulin expression in GRN-associated frontotemporal lobar degeneration. Abstract

Cairns, Nigel J

 Aston University 07/18/2009 9.32 Acta neuropathologica
TARDBP 3'-UTR variant in autopsy-confirmed frontotemporal lobar degeneration with TDP-43 proteinopathy. Abstract

Takahashi, Hitoshi

 Nishi-Niigata Chuo National Hospital 06/13/2009 9.32 Acta neuropathologica
Selective occurrence of TDP-43-immunoreactive inclusions in the lower motor neurons in Machado-Joseph disease. Abstract

Dickson, Dennis W

 Mayo Clinic 05/20/2009 9.32 Acta neuropathologica
Evaluation of subcortical pathology and clinical correlations in FTLD-U subtypes. Abstract

Wakabayashi, Koichi

 Hirosaki University Graduate School of Medicine 04/10/2009 9.32 Acta neuropathologica
Hippocampal sclerosis with four-repeat tau-positive round inclusions in the dentate gyrus: a new type of four-repeat tauopathy. Abstract

Mann, David M A

 University of Manchester 03/28/2009 9.32 Acta neuropathologica
TDP-43 in ubiquitinated inclusions in the inferior olives in frontotemporal lobar degeneration and in other neurodegenerative diseases: a degenerative process distinct from normal ageing. Abstract

Akiyama, Haruhiko

 Tokyo Institute of Psychiatry 02/05/2009 9.32 Acta neuropathologica
Clinicopathological characterization of Pick's disease versus frontotemporal lobar degeneration with ubiquitin/TDP-43-positive inclusions. Abstract

Akiyama, Haruhiko

 Tokyo Institute of Psychiatry 01/13/2009 9.32 Acta neuropathologica
Phosphorylated TDP-43 in Alzheimer's disease and dementia with Lewy bodies. Abstract

Lee, Virginia M-Y

 University of Pennsylvania School of Medicine 01/06/2009 9.32 Acta neuropathologica
Phosphorylation of S409/410 of TDP-43 is a consistent feature in all sporadic and familial forms of TDP-43 proteinopathies. Abstract

Takahashi, Hitoshi

 Nishi-Niigata Chuo National Hospital 10/16/2008 9.32 Acta neuropathologica
Sporadic amyotrophic lateral sclerosis of long duration is associated with relatively mild TDP-43 pathology. Abstract

Edbauer, Dieter

 German Center for Neurodegenerative Diseases (DZNE), Munich 12/19/2013 9.21 The EMBO journal
The FTLD risk factor TMEM106B and MAP6 control dendritic trafficking of lysosomes. Abstract

Xia, Xu-Gang

 Thomas Jefferson University 05/28/2013 9.21 The EMBO journal
Expression of ALS-linked TDP-43 mutant in astrocytes causes non-cell-autonomous motor neuron death in rats. Abstract

Haass, Christian

 Ludwig-Maximilians-University and German Center for Neurodegenerative Diseases (DZNE) Munich 09/11/2012 9.21 The EMBO journal
Arginine methylation next to the PY-NLS modulates Transportin binding and nuclear import of FUS. Abstract

Lee, Virginia M Y

 University of Pennsylvania School of Medicine 12/23/2011 9.21 The EMBO journal
Redox signalling directly regulates TDP-43 via cysteine oxidation and disulphide cross-linking. Abstract

Baralle, Francisco E

 International Centre for Genetic Engineering and Biotechnology (ICGEB), 34012 Trieste 12/03/2010 9.21 The EMBO journal
TDP-43 regulates its mRNA levels through a negative feedback loop. Abstract

Haass, Christian

 Ludwig-Maximilians-University and German Center for Neurodegenerative Diseases (DZNE) Munich 07/06/2010 9.21 The EMBO journal
ALS-associated fused in sarcoma (FUS) mutations disrupt Transportin-mediated nuclear import. Abstract

Parker, J Alex

 CRCHUM, Université de Montréal, Montréal, Québec 07/05/2012 8.69 PLoS genetics
TDP-1/TDP-43 regulates stress signaling and age-dependent proteotoxicity in Caenorhabditis elegans. Abstract

Drapeau, Pierre

 Department of Pathology and Cell Biology and Groupe de Recherche sur le Système Nerveux Central, Université de Montréal, Montréal, QC, … 08/04/2011 8.69 PLoS genetics
FUS and TARDBP but not SOD1 interact in genetic models of amyotrophic lateral sclerosis. Abstract

Xia, Xu-Gang

 Thomas Jefferson University 03/03/2011 8.69 PLoS genetics
FUS transgenic rats develop the phenotypes of amyotrophic lateral sclerosis and frontotemporal lobar degeneration. Abstract

Xia, Xu Gang

 Thomas Jefferson University 03/26/2010 8.69 PLoS genetics
Transgenic rat model of neurodegeneration caused by mutation in the TDP gene. Abstract

Shaw, Pamela J

 University of Sheffield 10/09/2013 8.31 Neurology
C9ORF72 transcription in a frontotemporal dementia case with two expanded alleles. Abstract

Grossman, Murray

 University of Pennsylvania School of Medicine 12/26/2012 8.31 Neurology
Can MRI screen for CSF biomarkers in neurodegenerative disease? Abstract

van den Berg, Leonard H

 University Medical Center Utrecht 07/25/2012 8.31 Neurology
Hexanucleotide repeat expansions in C9ORF72 in the spectrum of motor neuron diseases. Abstract

Suzuki, Norihiro

 Keio University 09/28/2011 8.31 Neurology
Conjoint pathologic cascades mediated by ALS/FTLD-U linked RNA-binding proteins TDP-43 and FUS. Abstract

Robberecht, W

 University Hospital Gasthuisberg 05/11/2011 8.31 Neurology
Expanded ATXN2 CAG repeat size in ALS identifies genetic overlap between ALS and SCA2. Abstract

Gitler, A D

 University of Pennsylvania School of Medicine 05/11/2011 8.31 Neurology
Evaluating the prevalence of polyglutamine repeat expansions in amyotrophic lateral sclerosis. Abstract

Sobue, G

 Nagoya University Graduate School of Medicine 03/16/2011 8.31 Neurology
Skin biopsy is useful for the antemortem diagnosis of neuronal intranuclear inclusion disease. Abstract

Rademakers, R

 Mayo Clinic College of Medicine 12/22/2010 8.31 Neurology
TMEM106B regulates progranulin levels and the penetrance of FTLD in GRN mutation carriers. Abstract

Josephs, K A

 Mayo Clinic 12/21/2010 8.31 Neurology
Does TDP-43 type confer a distinct pattern of atrophy in frontotemporal lobar degeneration? Abstract

Trojanowski, J Q

 University of Pennsylvania School of Medicine 11/03/2010 8.31 Neurology
Novel CSF biomarkers for frontotemporal lobar degenerations. Abstract

Grossman, M

 Hospital of the University of Pennsylvania 09/07/2010 8.31 Neurology
Distinct cerebral perfusion patterns in FTLD and AD. Abstract

Grossman, M

 Hospital of the University of Pennsylvania 08/17/2010 8.31 Neurology
Multimodal predictors for Alzheimer disease in nonfluent primary progressive aphasia. Abstract

Siddique, T

 Northwestern University Feinberg School of Medicine 07/28/2010 8.31 Neurology
Frameshift and novel mutations in FUS in familial amyotrophic lateral sclerosis and ALS/dementia. Abstract

Robberecht, W

 University Hospital Gasthuisberg 05/25/2010 8.31 Neurology
Tau levels do not influence human ALS or motor neuron degeneration in the SOD1G93A mouse. Abstract

Van Broeckhoven, C

 University of Antwerp-CDE 02/03/2010 8.31 Neurology
Genetic contribution of FUS to frontotemporal lobar degeneration. Abstract

Landers, J E

 University of Massachusetts Medical School 09/09/2009 8.31 Neurology
Analysis of FUS gene mutation in familial amyotrophic lateral sclerosis within an Italian cohort. Abstract

Rouleau, G A

 Notre-Dame Hospital 09/09/2009 8.31 Neurology
Mutations in FUS cause FALS and SALS in French and French Canadian populations. Abstract

Van Broeckhoven, C

 University of Antwerp-CDE 08/25/2009 8.31 Neurology
Clinical heterogeneity in 3 unrelated families linked to VCP p.Arg159His. Abstract

Siddique, T

 Northwestern University Feinberg School of Medicine 01/28/2009 8.31 Neurology
Age and founder effect of SOD1 A4V mutation causing ALS. Abstract

Baralle, Francisco E

 International Centre for Genetic Engineering and Biotechnology (ICGEB), 34012 Trieste 12/24/2013 8.03 Nucleic acids research
Predominance of spliceosomal complex formation over polyadenylation site selection in TDP-43 autoregulation. Abstract

Baralle, Francisco E

 International Centre for Genetic Engineering and Biotechnology (ICGEB), 34012 Trieste 03/21/2013 8.03 Nucleic acids research
Characterizing TDP-43 interaction with its RNA targets. Abstract

Baralle, Francisco E

 International Centre for Genetic Engineering and Biotechnology (ICGEB), 34012 Trieste 05/08/2009 8.03 Nucleic acids research
Functional mapping of the interaction between TDP-43 and hnRNP A2 in vivo. Abstract

Baloh, Robert H

 Washington University School of Medicine 10/24/2013 7.80 Science translational medicine
Targeting RNA foci in iPSC-derived motor neurons from ALS patients with a C9ORF72 repeat expansion. Abstract

Petrucelli, Leonard

 Mayo Clinic 10/26/2013 7.64 Human molecular genetics
Targeted manipulation of the sortilin-progranulin axis rescues progranulin haploinsufficiency. Abstract

Baloh, Robert H

 Washington University School of Medicine 08/19/2013 7.64 Human molecular genetics
Prion-like nuclear aggregation of TDP-43 during heat shock is regulated by HSP40/70 chaperones. Abstract

Van Broeckhoven, Christine

 Neurodegenerative Brain Diseases Group, Department of Molecular Genetics, VIB, Universiteitsplein 1, B-2610 Antwerpen 07/29/2013 7.64 Human molecular genetics
Pathological mechanisms underlying TDP-43 driven neurodegeneration in FTLD-ALS spectrum disorders. Abstract

Drapeau, Pierre

 Department of Pathology and Cell Biology and Groupe de Recherche sur le Système Nerveux Central, Université de Montréal, Montréal, QC, … 06/13/2013 7.64 Human molecular genetics
Loss and gain of FUS function impair neuromuscular synaptic transmission in a genetic model of ALS. Abstract

Petrucelli, Leonard

 Mayo Clinic 04/10/2013 7.64 Human molecular genetics
The dual functions of the extreme N-terminus of TDP-43 in regulating its biological activity and inclusion formation. Abstract

Shaw, Christopher E

 King's College London 03/07/2013 7.64 Human molecular genetics
ALS mutant FUS disrupts nuclear localization and sequesters wild-type FUS within cytoplasmic stress granules. Abstract

Shaw, Pamela J

 University of Sheffield 02/19/2013 7.64 Human molecular genetics
Tardbpl splicing rescues motor neuron and axonal development in a mutant tardbp zebrafish. Abstract

Parker, J Alex

 CRCHUM, Université de Montréal, Montréal, Québec 11/19/2012 7.64 Human molecular genetics
Reduction of polyglutamine toxicity by TDP-43, FUS and progranulin in Huntington's disease models. Abstract

Tibbetts, Randal S

 University of Wisconsin School of Medicine and Public Health 08/07/2012 7.64 Human molecular genetics
High-content RNAi screening identifies the Type 1 inositol triphosphate receptor as a modifier of TDP-43 localization and neurotoxicity. Abstract

Xia, Xu-Gang

 Thomas Jefferson University 07/23/2012 7.64 Human molecular genetics
Entorhinal cortical neurons are the primary targets of FUS mislocalization and ubiquitin aggregation in FUS transgenic rats. Abstract

van den Berg, Leonard H

 University Medical Center Utrecht 05/29/2012 7.64 Human molecular genetics
Evidence for an oligogenic basis of amyotrophic lateral sclerosis. Abstract

Petrucelli, Leonard

 Mayo Clinic 04/05/2012 7.64 Human molecular genetics
Loss of HDAC6, a novel CHIP substrate, alleviates abnormal tau accumulation. Abstract

Gitler, Aaron D

 Stanford University School of Medicine 03/27/2012 7.64 Human molecular genetics
Evaluating the role of the FUS/TLS-related gene EWSR1 in amyotrophic lateral sclerosis. Abstract

Rademakers, Rosa

 Mayo Clinic 05/24/2011 7.64 Human molecular genetics
Ataxin-2 repeat-length variation and neurodegeneration. Abstract

Gitler, Aaron D

 Stanford University School of Medicine 02/03/2011 7.64 Human molecular genetics
Ataxin-2 intermediate-length polyglutamine expansions in European ALS patients. Abstract

Cleveland, Don W

 University of California at San Diego 04/15/2010 7.64 Human molecular genetics
TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration. Abstract

Drapeau, Pierre

 Department of Pathology and Cell Biology and Groupe de Recherche sur le Système Nerveux Central, Université de Montréal, Montréal, QC, … 12/03/2009 7.64 Human molecular genetics
Gain and loss of function of ALS-related mutations of TARDBP (TDP-43) cause motor deficits in vivo. Abstract

Trojanowski, John Q

 University of Pennsylvania School of Medicine 10/07/2009 7.64 Human molecular genetics
Mutations in TDP-43 link glycine-rich domain functions to amyotrophic lateral sclerosis. Abstract

Rouleau, Guy A

 Centre of Excellence in Neurosciences of Université de Montréal, CHUM Research Center, Montreal, Québec 11/01/2012 7.58 Archives of neurology
Novel FUS deletion in a patient with juvenile amyotrophic lateral sclerosis. Abstract

Grossman, Murray

 University of Pennsylvania 08/22/2012 7.58 Archives of neurology
Comparison of cerebrospinal fluid levels of tau and Aβ 1-42 in Alzheimer disease and frontotemporal degeneration using 2 analytical platforms. Abstract

Rouleau, Guy A

 Centre of Excellence in Neurosciences of Université de Montréal, CHUM Research Center, Montreal, QC H2L 4M1 06/14/2011 7.58 Archives of neurology
Association of long ATXN2 CAG repeat sizes with increased risk of amyotrophic lateral sclerosis. Abstract

Trojanowski, John Q

 University of Pennsylvania School of Medicine 10/12/2010 7.58 Archives of neurology
Pathological 43-kDa transactivation response DNA-binding protein in older adults with and without severe mental illness. Abstract

Shaw, Pamela J

 University of Sheffield 04/13/2010 7.58 Archives of neurology
Novel FUS/TLS mutations and pathology in familial and sporadic amyotrophic lateral sclerosis. Abstract

van den Berg, Leonard H

 University Medical Center Utrecht 02/09/2010 7.58 Archives of neurology
FUS mutations in familial amyotrophic lateral sclerosis in the Netherlands. Abstract

Shen, Che-Kun James

 Institute of Life Science 10/29/2012 7.45 Autophagy
Autophagy activation ameliorates neuronal pathogenesis of FTLD-U mice: a new light for treatment of TARDBP/TDP-43 proteinopathies. Abstract

Cleveland, Don W

 University of California at San Diego 08/02/2011 7.44 Current opinion in neurobiology
Understanding the role of TDP-43 and FUS/TLS in ALS and beyond. Abstract

Kwak, Shin

 The University of Tokyo 12/19/2012 7.40 Nature communications
A role for calpain-dependent cleavage of TDP-43 in amyotrophic lateral sclerosis pathology. Abstract

Lee, Virginia M Y

 University of Pennsylvania School of Medicine 03/23/2011 7.40 Nature communications
The acetylation of tau inhibits its function and promotes pathological tau aggregation. Abstract

Edbauer, Dieter

 German Center for Neurodegenerative Diseases (DZNE), Munich 06/19/2012 7.36 EMBO reports
Loss of fused in sarcoma (FUS) promotes pathological Tau splicing. Abstract

Mackenzie, Ian R A

 University Hospital of Zürich 07/29/2009 7.14 Expert reviews in molecular medicine
The molecular basis of frontotemporal dementia. Abstract

Drapeau, Pierre

 Department of Pathology and Cell Biology and Groupe de Recherche sur le Système Nerveux Central, Université de Montréal, Montréal, QC, … 01/24/2013 7.12 The Journal of neuroscience : the official journal of the Society for Neuroscience
Calcium channel agonists protect against neuromuscular dysfunction in a genetic model of TDP-43 mutation in ALS. Abstract

Oddo, Salvatore

 University of Texas Health Science Center at San Antonio 01/17/2013 7.12 The Journal of neuroscience : the official journal of the Society for Neuroscience
Glucocorticoids exacerbate cognitive deficits in TDP-25 transgenic mice via a glutathione-mediated mechanism: implications for aging, stress and TDP-43 proteinopathies. Abstract

Julien, Jean-Pierre

 Department of Psychiatry and Neuroscience of Laval University 12/14/2012 7.12 The Journal of neuroscience : the official journal of the Society for Neuroscience
Abnormal regenerative responses and impaired axonal outgrowth after nerve crush in TDP-43 transgenic mouse models of amyotrophic lateral sclerosis. Abstract

Lee, Virginia M-Y

 University of Pennsylvania School of Medicine 08/16/2012 7.12 The Journal of neuroscience : the official journal of the Society for Neuroscience
TMEM106B, the risk gene for frontotemporal dementia, is regulated by the microRNA-132/212 cluster and affects progranulin pathways. Abstract

Gitler, Aaron D

 Stanford University School of Medicine 07/05/2012 7.12 The Journal of neuroscience : the official journal of the Society for Neuroscience
ALS-associated ataxin 2 polyQ expansions enhance stress-induced caspase 3 activation and increase TDP-43 pathological modifications. Abstract

Lee, Virginia M-Y

 University of Pennsylvania School of Medicine 07/07/2011 7.12 The Journal of neuroscience : the official journal of the Society for Neuroscience
α-Syn suppression reverses synaptic and memory defects in a mouse model of dementia with Lewy bodies. Abstract

Haass, Christian

 Ludwig-Maximilians-University and German Center for Neurodegenerative Diseases (DZNE) Munich 02/03/2011 7.12 The Journal of neuroscience : the official journal of the Society for Neuroscience
Rescue of progranulin deficiency associated with frontotemporal lobar degeneration by alkalizing reagents and inhibition of vacuolar ATPase. Abstract

Kwak, Shin

 University of Tokyo 09/09/2010 7.12 The Journal of neuroscience : the official journal of the Society for Neuroscience
Induced loss of ADAR2 engenders slow death of motor neurons from Q/R site-unedited GluR2. Abstract

Petrucelli, Leonard

 Mayo Clinic 08/12/2010 7.12 The Journal of neuroscience : the official journal of the Society for Neuroscience
Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice. Abstract

Klein, Ronald L

 Louisiana State University Health Sciences Center 05/21/2013 6.87 Molecular therapy : the journal of the American Society of Gene Therapy
Selective forelimb impairment in rats expressing a pathological TDP-43 25 kDa C-terminal fragment to mimic amyotrophic lateral sclerosis. Abstract

Klein, Ronald L

 Louisiana State University Health Sciences Center 09/28/2010 6.87 Molecular therapy : the journal of the American Society of Gene Therapy
Expansive gene transfer in the rat CNS rapidly produces amyotrophic lateral sclerosis relevant sequelae when TDP-43 is overexpressed. Abstract

Klein, Ronald L

 Louisiana State University Health Sciences Center 02/17/2009 6.87 Molecular therapy : the journal of the American Society of Gene Therapy
Mimicking aspects of frontotemporal lobar degeneration and Lou Gehrig's disease in rats via TDP-43 overexpression. Abstract

Rouleau, G A

 Notre-Dame Hospital 10/17/2008 6.37 Journal of medical genetics
Contribution of TARDBP mutations to sporadic amyotrophic lateral sclerosis. Abstract

Kumar-Singh, Samir

 Neurodegenerative Brain Diseases Group, Department of Molecular Genetics, VIB, Universiteitsplein 1, 2610, Antwerp 06/25/2012 6.32 The Journal of pathology
Cellular ageing, increased mortality and FTLD-TDP-associated neuropathology in progranulin knockout mice. Abstract

Parker, J Alex

 CRCHUM, Université de Montréal, Montréal, Québec 08/01/2012 6.27 Aging cell
Glucose delays age-dependent proteotoxicity. Abstract

Grossman, Murray

 University of Pennsylvania School of Medicine 12/02/2013 6.19 Neurobiology of aging
Genetic and neuroanatomic associations in sporadic frontotemporal lobar degeneration. Abstract

Gitler, Aaron D

 Stanford University School of Medicine 10/23/2013 6.19 Neurobiology of aging
Evaluating noncoding nucleotide repeat expansions in amyotrophic lateral sclerosis. Abstract

Hardiman, Orla

 Trinity College Dublin 08/21/2013 6.19 Neurobiology of aging
UBQLN2 mutations are not a frequent cause of amyotrophic lateral sclerosis in Ireland. Abstract

Oddo, Salvatore

 University of Texas Health Science Center at San Antonio 08/15/2013 6.19 Neurobiology of aging
Accumulation of C-terminal fragments of transactive response DNA-binding protein 43 leads to synaptic loss and cognitive deficits in human TDP-43 transgenic mice. Abstract

Revesz, Tamas

 University College London 05/09/2013 6.19 Neurobiology of aging
TDP-43 pathology in a patient carrying G2019S LRRK2 mutation and a novel p.Q124E MAPT. Abstract

Rademakers, Rosa

 Mayo Clinic 04/28/2013 6.19 Neurobiology of aging
Mutations in protein N-arginine methyltransferases are not the cause of FTLD-FUS. Abstract

Blair, Ian P

 ANZAC Research Institute 04/28/2013 6.19 Neurobiology of aging
Mutation analysis and immunopathological studies of PFN1 in familial and sporadic amyotrophic lateral sclerosis. Abstract

Parker, J Alex

 CRCHUM, Université de Montréal, Montréal, Québec 04/13/2013 6.19 Neurobiology of aging
Evaluation of longevity enhancing compounds against transactive response DNA-binding protein-43 neuronal toxicity. Abstract

Rouleau, Guy A

 Centre of Excellence in Neurosciences of Université de Montréal, CHUM Research Center, Montreal, Québec 10/10/2012 6.19 Neurobiology of aging
Mutation analysis of PFN1 in familial amyotrophic lateral sclerosis patients. Abstract

van den Berg, Leonard H

 University Medical Center Utrecht 10/09/2012 6.19 Neurobiology of aging
Mutational analysis of TARDBP in Parkinson's disease. Abstract

Shaw, Christopher E

 King's College London 08/11/2012 6.19 Neurobiology of aging
Screening for OPTN mutations in a cohort of British amyotrophic lateral sclerosis patients. Abstract

van den Berg, Leonard H

 University Medical Center Utrecht 08/09/2012 6.19 Neurobiology of aging
VAPB and C9orf72 mutations in 1 familial amyotrophic lateral sclerosis patient. Abstract

Rademakers, Rosa

 Mayo Clinic 07/26/2012 6.19 Neurobiology of aging
Length of normal alleles of C9ORF72 GGGGCC repeat do not influence disease phenotype. Abstract

Strong, Michael J

 Robarts Research Institute 07/24/2012 6.19 Neurobiology of aging
Rho guanine nucleotide exchange factor is an NFL mRNA destabilizing factor that forms cytoplasmic inclusions in amyotrophic lateral sclerosis. Abstract

Blair, Ian P

 ANZAC Research Institute 06/19/2012 6.19 Neurobiology of aging
UBQLN2/ubiquilin 2 mutation and pathology in familial amyotrophic lateral sclerosis. Abstract

van den Berg, Leonard H

 University Medical Center Utrecht 06/05/2012 6.19 Neurobiology of aging
UBQLN2 in familial amyotrophic lateral sclerosis in The Netherlands. Abstract

Traynor, Bryan J

 National Institute on Aging 05/08/2012 6.19 Neurobiology of aging
Valosin-containing protein (VCP) mutations in sporadic amyotrophic lateral sclerosis. Abstract

Rouleau, Guy A

 Centre of Excellence in Neurosciences of Université de Montréal, CHUM Research Center, Montreal, Québec 05/03/2012 6.19 Neurobiology of aging
UBQLN2 mutations are rare in French and French-Canadian amyotrophic lateral sclerosis. Abstract

Rouleau, Guy A

 Centre of Excellence in Neurosciences of Université de Montréal, CHUM Research Center, Montreal, QC H2L 4M1 02/22/2012 6.19 Neurobiology of aging
Analysis of the SORT1 gene in familial amyotrophic lateral sclerosis. Abstract

van den Berg, Leonard H

 University Medical Center Utrecht 02/11/2012 6.19 Neurobiology of aging
Rare and common paraoxonase gene variants in amyotrophic lateral sclerosis patients. Abstract

Blair, Ian P

 ANZAC Research Institute 12/22/2011 6.19 Neurobiology of aging
Mutation analysis of VCP in familial and sporadic amyotrophic lateral sclerosis. Abstract

van den Berg, Leonard H

 University Medical Center Utrecht 11/10/2011 6.19 Neurobiology of aging
VCP mutations in familial and sporadic amyotrophic lateral sclerosis. Abstract

Van Broeckhoven, Christine

 Neurodegenerative Brain Diseases Group, Department of Molecular Genetics, VIB, Universiteitsplein 1, B-2610 Antwerpen 10/27/2011 6.19 Neurobiology of aging
Ataxin-2 polyQ expansions in FTLD-ALS spectrum disorders in Flanders-Belgian cohorts. Abstract

Blair, Ian P

 ANZAC Research Institute 10/19/2011 6.19 Neurobiology of aging
Mutation analysis of the optineurin gene in familial amyotrophic lateral sclerosis. Abstract

van den Berg, Leonard H

 University Medical Center Utrecht 07/28/2011 6.19 Neurobiology of aging
Novel optineurin mutations in sporadic amyotrophic lateral sclerosis patients. Abstract

Kawakami, Hideshi

 Hiroshima University 05/06/2011 6.19 Neurobiology of aging
Screening for OPTN mutations in amyotrophic lateral sclerosis in a mainly Caucasian population. Abstract

Rademakers, Rosa

 Mayo Clinic 11/12/2010 6.19 Neurobiology of aging
Pathogenicity of exonic indels in fused in sarcoma in amyotrophic lateral sclerosis. Abstract

Rouleau, Guy A

 Centre of Excellence in Neurosciences of Université de Montréal, CHUM Research Center, Montreal, Québec 11/11/2010 6.19 Neurobiology of aging
Analysis of OPTN as a causative gene for amyotrophic lateral sclerosis. Abstract

Traynor, Bryan J

 National Institute on Aging 02/06/2010 6.19 Neurobiology of aging
FUS mutations in sporadic amyotrophic lateral sclerosis. Abstract

Landers, John E

 University of Massachusetts Medical School 12/23/2009 6.19 Neurobiology of aging
Mutational analysis of TARDBP in neurodegenerative diseases. Abstract

Josephs, Keith A

 Mayo Clinic 11/14/2009 6.19 Neurobiology of aging
Temporoparietal atrophy: a marker of AD pathology independent of clinical diagnosis. Abstract

Julien, Jean-Pierre

 Department of Psychiatry and Neuroscience of Laval University 09/26/2009 6.19 Neurobiology of aging
Gigaxonin mutation analysis in patients with NIFID. Abstract

Kumar-Singh, Samir

 Neurodegenerative Brain Diseases Group, Department of Molecular Genetics, VIB, Universiteitsplein 1, 2610, Antwerp 03/10/2013 5.74 Molecular neurobiology
Overexpression of ALS-associated p.M337V human TDP-43 in mice worsens disease features compared to wild-type human TDP-43 mice. Abstract

Van Broeckhoven, Christine

 University of Antwerp-CDE 12/13/2012 5.74 Molecular neurobiology
Mechanisms of granulin deficiency: lessons from cellular and animal models. Abstract

Van Broeckhoven, Christine

 University of Antwerp-CDE 07/02/2012 5.69 Human mutation
Locus-specific mutation databases for neurodegenerative brain diseases. Abstract

Rademakers, Rosa

 Mayo Clinic 05/03/2010 5.69 Human mutation
De novo truncating FUS gene mutation as a cause of sporadic amyotrophic lateral sclerosis. Abstract

Shaw, Pamela J

 University of Sheffield 12/04/2009 5.42 Free radical biology & medicine
Oxidative stress in ALS: key role in motor neuron injury and therapeutic target. Abstract

Parker, J Alex

 CRCHUM, Université de Montréal, Montréal, Québec 04/05/2013 5.40 Neurobiology of disease
Pharmacological reduction of ER stress protects against TDP-43 neuronal toxicity in vivo. Abstract

Kwak, Shin

 University of Tokyo 12/28/2011 5.40 Neurobiology of disease
Profound downregulation of the RNA editing enzyme ADAR2 in ALS spinal motor neurons. Abstract

Sobue, Gen

 Nagoya University Graduate School of Medicine 12/13/2011 5.40 Neurobiology of disease
Oxidative stress induced by glutathione depletion reproduces pathological modifications of TDP-43 linked to TDP-43 proteinopathies. Abstract

Cairns, N J

 Aston University 09/10/2011 5.40 Neurobiology of disease
Core features of frontotemporal dementia recapitulated in progranulin knockout mice. Abstract

Julien, Jean-Pierre

 Department of Psychiatry and Neuroscience of Laval University 06/02/2010 5.40 Neurobiology of disease
Wild-type human SOD1 overexpression does not accelerate motor neuron disease in mice expressing murine Sod1 G86R. Abstract

Baloh, Robert H

 Washington University School of Medicine 11/19/2012 4.94 Current opinion in neurology
How do the RNA-binding proteins TDP-43 and FUS relate to amyotrophic lateral sclerosis and frontotemporal degeneration, and to each other? Abstract

Josephs, Keith A

 Mayo Clinic 07/08/2010 4.94 Current opinion in neurology
Neuropathology of variants of progressive supranuclear palsy. Abstract

Robberecht, Wim

 VIB Vesalius Research Center, Leuven 09/16/2009 4.94 Current opinion in neurology
Recent advances in motor neuron disease. Abstract

Baralle, Francisco Ernesto

 International Centre for Genetic Engineering and Biotechnology, Trieste 07/01/2010 4.93 RNA biology
The multiple roles of TDP-43 in pre-mRNA processing and gene expression regulation. Abstract

Trojanowski, John Q

 University of Pennsylvania School of Medicine 07/26/2013 4.89 The American journal of pathology
Acetylated tau neuropathology in sporadic and hereditary tauopathies. Abstract

Oddo, Salvatore

 University of Texas Health Science Center at San Antonio 11/07/2011 4.89 The American journal of pathology
Cognitive decline typical of frontotemporal lobar degeneration in transgenic mice expressing the 25-kDa C-terminal fragment of TDP-43. Abstract

Lee, Virginia M-Y

 University of Pennsylvania School of Medicine 08/27/2010 4.89 The American journal of pathology
A{beta} accelerates the spatiotemporal progression of tau pathology and augments tau amyloidosis in an Alzheimer mouse model. Abstract

Tibbetts, Randal S

 University of Wisconsin School of Medicine and Public Health 07/05/2013 4.77 The Journal of biological chemistry
The RNA-binding protein fused in sarcoma (FUS) functions downstream of poly(ADP-ribose) polymerase (PARP) in response to DNA damage. Abstract

Suzuki, Norihiro

 Keio University 10/09/2012 4.77 The Journal of biological chemistry
Roles of ataxin-2 in pathological cascades mediated by TAR DNA-binding protein 43 (TDP-43) and Fused in Sarcoma (FUS). Abstract

Shen, C-K James

 Institute of Molecular Biology 06/20/2012 4.77 The Journal of biological chemistry
Targeted depletion of TDP-43 expression in the spinal cord motor neurons leads to the development of amyotrophic lateral sclerosis-like phenotypes in mice. Abstract

Haass, Christian

 Ludwig-Maximilians-University and German Center for Neurodegenerative Diseases (DZNE) Munich 05/04/2012 4.77 The Journal of biological chemistry
Requirements for stress granule recruitment of fused in sarcoma (FUS) and TAR DNA-binding protein of 43 kDa (TDP-43). Abstract

Haass, Christian

 Ludwig-Maximilians-University and German Center for Neurodegenerative Diseases (DZNE) Munich 04/17/2012 4.77 The Journal of biological chemistry
Membrane orientation and subcellular localization of transmembrane protein 106B (TMEM106B), a major risk factor for frontotemporal lobar degeneration. Abstract

Baralle, Francisco E

 International Centre for Genetic Engineering and Biotechnology (ICGEB), 34012 Trieste 01/10/2012 4.77 The Journal of biological chemistry
Cellular model of TAR DNA-binding protein 43 (TDP-43) aggregation based on its C-terminal Gln/Asn-rich region. Abstract

Shen, C-K James

 Institute of Molecular Biology 11/03/2011 4.77 The Journal of biological chemistry
Regulation of autophagy by neuropathological protein TDP-43. Abstract

Lee, Virginia M-Y

 University of Pennsylvania School of Medicine 03/24/2011 4.77 The Journal of biological chemistry
A "two-hit" hypothesis for inclusion formation by carboxyl-terminal fragments of TDP-43 protein linked to RNA depletion and impaired microtubule-dependent transport. Abstract

Tibbetts, Randal S

 University of Wisconsin School of Medicine and Public Health 08/18/2010 4.77 The Journal of biological chemistry
Amyotrophic lateral sclerosis-associated proteins TDP-43 and FUS/TLS function in a common biochemical complex to co-regulate HDAC6 mRNA. Abstract

Baloh, Robert H

 Washington University School of Medicine 06/16/2010 4.77 The Journal of biological chemistry
Interaction with polyglutamine aggregates reveals a Q/N-rich domain in TDP-43. Abstract

Tibbetts, Randal S

 University of Wisconsin School of Medicine and Public Health 02/12/2010 4.77 The Journal of biological chemistry
Ubiquilin modifies TDP-43 toxicity in a Drosophila model of amyotrophic lateral sclerosis (ALS). Abstract

Suzuki, Norihiro

 Keio University 11/03/2009 4.77 The Journal of biological chemistry
Characterization of alternative isoforms and inclusion body of the TAR DNA-binding protein-43. Abstract

Oddo, Salvatore

 University of Texas Health Science Center at San Antonio 08/03/2009 4.77 The Journal of biological chemistry
Rapamycin rescues TDP-43 mislocalization and the associated low molecular mass neurofilament instability. Abstract

Sobue, Gen

 Nagoya University Graduate School of Medicine 06/17/2009 4.77 The Journal of biological chemistry
TDP-43 depletion induces neuronal cell damage through dysregulation of Rho family GTPases. Abstract

Gitler, Aaron D

 Stanford University School of Medicine 05/22/2009 4.77 The Journal of biological chemistry
TDP-43 is intrinsically aggregation-prone, and amyotrophic lateral sclerosis-linked mutations accelerate aggregation and increase toxicity. Abstract

Cairns, Nigel J

 Aston University 02/23/2009 4.77 The Journal of biological chemistry
VCP mutations causing frontotemporal lobar degeneration disrupt localization of TDP-43 and induce cell death. Abstract

Lee, Virginia M-Y

 University of Pennsylvania School of Medicine 01/21/2009 4.77 The Journal of biological chemistry
Expression of TDP-43 C-terminal Fragments in Vitro Recapitulates Pathological Features of TDP-43 Proteinopathies. Abstract

Tibbetts, Randal S

 University of Wisconsin School of Medicine and Public Health 12/26/2008 4.77 The Journal of biological chemistry
Potentiation of amyotrophic lateral sclerosis (ALS)-associated TDP-43 aggregation by the proteasome-targeting factor, ubiquilin 1. Abstract

Grossman, Murray

 University of Pennsylvania School of Medicine 03/09/2013 4.76 Journal of neurology, neurosurgery, and psychiatry
White matter imaging helps dissociate tau from TDP-43 in frontotemporal lobar degeneration. Abstract

Grossman, Murray

 University of Pennsylvania School of Medicine 10/31/2012 4.76 Journal of neurology, neurosurgery, and psychiatry
Cognitive decline and reduced survival in C9orf72 expansion frontotemporal degeneration and amyotrophic lateral sclerosis. Abstract

Shaw, Pamela J

 University of Sheffield 10/20/2012 4.76 Journal of neurology, neurosurgery, and psychiatry
Concurrence of multiple sclerosis and amyotrophic lateral sclerosis in patients with hexanucleotide repeat expansions of C9ORF72. Abstract

Trojanowski, John Q

 University of Pennsylvania School of Medicine 10/20/2012 4.76 Journal of neurology, neurosurgery, and psychiatry
Therapeutic strategies for tau mediated neurodegeneration. Abstract

van den Berg, Leonard H

 University Medical Center Utrecht 09/29/2011 4.76 Journal of neurology, neurosurgery, and psychiatry
Structural MRI reveals cortical thinning in amyotrophic lateral sclerosis. Abstract

Van den Berg, L H

 University Medical Centre Utrecht 04/06/2011 4.76 Journal of neurology, neurosurgery, and psychiatry
Beneficial vascular risk profile is associated with amyotrophic lateral sclerosis. Abstract

Hardiman, Orla

 Trinity College Dublin 11/03/2010 4.76 Journal of neurology, neurosurgery, and psychiatry
Rate of familial amyotrophic lateral sclerosis: a systematic review and meta-analysis. Abstract

Rademakers, Rosa

 Mayo Clinic 06/20/2010 4.76 Journal of neurology, neurosurgery, and psychiatry
Clinical, neuroimaging and neuropathological features of a new chromosome 9p-linked FTD-ALS family. Abstract

Sobue, Gen

 Nagoya University Graduate School of Medicine 12/03/2009 4.76 Journal of neurology, neurosurgery, and psychiatry
The profile of motor unit number estimation (MUNE) in spinal and bulbar muscular atrophy. Abstract

Blair, I P

 Concord Hospital NSW 2139 10/29/2009 4.76 Journal of neurology, neurosurgery, and psychiatry
A novel TARDBP mutation in an Australian amyotrophic lateral sclerosis kindred. Abstract

Klein, Ronald L

 Louisiana State University Health Sciences Center 12/09/2011 4.70 Experimental neurology
Frontotemporal lobar degeneration-related proteins induce only subtle memory-related deficits when bilaterally overexpressed in the dorsal hippocampus. Abstract

Wakabayashi, Koichi

 Hirosaki University Graduate School of Medicine 01/18/2010 4.70 Experimental neurology
Alteration of biochemical and pathological properties of TDP-43 protein by a lipid mediator, 15-deoxy-Delta(12,14)-prostaglandin J(2). Abstract

Edbauer, Dieter

 German Center for Neurodegenerative Diseases (DZNE), Munich 08/24/2013 4.67 Journal of molecular medicine (Berlin, Germany)
FUS-mediated alternative splicing in the nervous system: consequences for ALS and FTLD. Abstract

Blair, Ian P

 ANZAC Research Institute 06/25/2010 4.63 The international journal of biochemistry & cell biology
TDP-43: a DNA and RNA binding protein with roles in neurodegenerative diseases. Abstract

Blair, Ian P

 ANZAC Research Institute 06/10/2010 4.63 The international journal of biochemistry & cell biology
Fused in sarcoma/translocated in liposarcoma: a multifunctional DNA/RNA binding protein. Abstract

Josephs, Keith A

 Mayo Clinic 08/03/2009 4.51 Movement disorders : official journal of the Movement Disorder Society
Alzheimer's disease and corticobasal degeneration presenting as corticobasal syndrome. Abstract

Rouleau, Guy A

 Centre of Excellence in Neurosciences of Université de Montréal, CHUM Research Center, Montreal, Québec 06/27/2012 4.40 European journal of human genetics : EJHG
Genetic analysis of SIGMAR1 as a cause of familial ALS with dementia. Abstract

Shaw, Christopher E

 Institute of Psychiatry 06/13/2012 4.40 European journal of human genetics : EJHG
The C9ORF72 expansion mutation is a common cause of ALS+/-FTD in Europe and has a single founder. Abstract

Bosco, Daryl A

 University of Massachusetts Medical School 08/31/2013 4.28 Molecular neurodegeneration
Amyotrophic lateral sclerosis-linked FUS/TLS alters stress granule assembly and dynamics. Abstract

Petrucelli, Leonard

 Mayo Clinic 07/10/2012 4.28 Molecular neurodegeneration
Progranulin regulates neuronal outgrowth independent of sortilin. Abstract

Petrucelli, Leonard

 Mayo Clinic 10/26/2011 4.28 Molecular neurodegeneration
Expression of mutant TDP-43 induces neuronal dysfunction in transgenic mice. Abstract

Oddo, Salvatore

 University of Texas Health Science Center at San Antonio 11/11/2010 4.28 Molecular neurodegeneration
Age-dependent changes in TDP-43 levels in a mouse model of Alzheimer disease are linked to Aβ oligomers accumulation. Abstract

Petrucelli, Leonard

 Mayo Clinic 08/30/2010 4.28 Molecular neurodegeneration
Phosphorylation regulates proteasomal-mediated degradation and solubility of TAR DNA binding protein-43 C-terminal fragments. Abstract

Wakabayashi, Koichi

 Hirosaki University Graduate School of Medicine 12/24/2012 4.26 Journal of neuropathology and experimental neurology
Keap1 is localized in neuronal and glial cytoplasmic inclusions in various neurodegenerative diseases. Abstract

Wakabayashi, Koichi

 Hirosaki University Graduate School of Medicine 09/22/2011 4.26 Journal of neuropathology and experimental neurology
Synphilin-1-binding protein NUB1 is colocalized with nonfibrillar, proteinase K-resistant α-synuclein in presynapses in Lewy body disease. Abstract

Wakabayashi, Koichi

 Hirosaki University Graduate School of Medicine 11/10/2009 4.26 Journal of neuropathology and experimental neurology
Decreased cystatin C immunoreactivity in spinal motor neurons and astrocytes in amyotrophic lateral sclerosis. Abstract

Dickson, Dennis W

 Mayo Clinic 11/10/2009 4.26 Journal of neuropathology and experimental neurology
Transactivation response DNA-binding protein 43 microvasculopathy in frontotemporal degeneration and familial Lewy body disease. Abstract

Parker, J Alex

 CRCHUM, Université de Montréal, Montréal, Québec 12/12/2013 4.09 PloS one
Deletion of C9ORF72 results in motor neuron degeneration and stress sensitivity in C. elegans. Abstract

Sobue, Gen

 Nagoya University Graduate School of Medicine 06/28/2013 4.09 PloS one
RNP2 of RNA recognition motif 1 plays a central role in the aberrant modification of TDP-43. Abstract

Landers, John E

 University of Massachusetts Medical School 04/08/2013 4.09 PloS one
Characterization of FUS mutations in amyotrophic lateral sclerosis using RNA-Seq. Abstract

Tibbetts, Randal S

 University of Wisconsin School of Medicine and Public Health 02/27/2013 4.09 PloS one
Identification of genetic modifiers of TDP-43 neurotoxicity in Drosophila. Abstract

Sobue, Gen

 Nagoya University Graduate School of Medicine 02/07/2013 4.09 PloS one
dnc-1/dynactin 1 knockdown disrupts transport of autophagosomes and induces motor neuron degeneration. Abstract

van den Berg, Leonard H

 University Medical Center Utrecht 11/14/2012 4.09 PloS one
Genetic overlap between apparently sporadic motor neuron diseases. Abstract

Sobue, Gen

 Nagoya University Graduate School of Medicine 09/25/2012 4.09 PloS one
c-Abl inhibition delays motor neuron degeneration in the G93A mouse, an animal model of amyotrophic lateral sclerosis. Abstract

Kwak, Shin

 The University of Tokyo 08/20/2012 4.09 PloS one
Co-occurrence of TDP-43 mislocalization with reduced activity of an RNA editing enzyme, ADAR2, in aged mouse motor neurons. Abstract

Parker, J Alex

 CRCHUM, Université de Montréal, Montréal, Québec 07/27/2012 4.09 PloS one
Methylene blue protects against TDP-43 and FUS neuronal toxicity in C. elegans and D. rerio. Abstract

Julien, Jean-Pierre

 Department of Psychiatry and Neuroscience of Laval University 04/16/2012 4.09 PloS one
Ablation of proliferating cells in the CNS exacerbates motor neuron disease caused by mutant superoxide dismutase. Abstract

Parker, J Alex

 CRCHUM, Université de Montréal, Montréal, Québec 02/21/2012 4.09 PloS one
Mutant TDP-43 and FUS cause age-dependent paralysis and neurodegeneration in C. elegans. Abstract

Shaw, Pamela J

 University of Sheffield 08/18/2011 4.09 PloS one
Optimised and rapid pre-clinical screening in the SOD1(G93A) transgenic mouse model of amyotrophic lateral sclerosis (ALS). Abstract

Cleveland, Don W

 University of California at San Diego 07/11/2011 4.09 PloS one
Misfolded SOD1 associated with motor neuron mitochondria alters mitochondrial shape and distribution prior to clinical onset. Abstract

Shen, C-K James

 Institute of Molecular Biology 06/01/2011 4.09 PloS one
Neuronal function and dysfunction of Drosophila dTDP. Abstract

Bonini, Nancy M

 University of Pennsylvania 03/29/2011 4.09 PloS one
PolyQ repeat expansions in ATXN2 associated with ALS are CAA interrupted repeats. Abstract

van den Berg, Leonard H

 University Medical Center Utrecht 10/27/2010 4.09 PloS one
Motor network degeneration in amyotrophic lateral sclerosis: a structural and functional connectivity study. Abstract

Robberecht, Wim

 VIB Vesalius Research Center, Leuven 10/13/2010 4.09 PloS one
Progranulin is neurotrophic in vivo and protects against a mutant TDP-43 induced axonopathy. Abstract

Shaw, Pamela J

 University of Sheffield 03/24/2010 4.09 PloS one
Mutations in CHMP2B in lower motor neuron predominant amyotrophic lateral sclerosis (ALS). Abstract

Rademakers, Rosa

 Mayo Clinic 10/27/2011 4.07 BMC genomics
Altered microRNA expression in frontotemporal lobar degeneration with TDP-43 pathology caused by progranulin mutations. Abstract

Rademakers, Rosa

 Mayo Clinic 07/01/2013 4.06 Journal of neurochemistry
TMEM106B p.T185S regulates TMEM106B protein levels: implications for frontotemporal dementia. Abstract

Julien, Jean-Pierre

 Department of Psychiatry and Neuroscience of Laval University 10/26/2010 4.06 Journal of neurochemistry
Neuronal over-expression of chromogranin A accelerates disease onset in a mouse model of ALS. Abstract

Kumar-Singh, Samir

 Neurodegenerative Brain Diseases Group, Department of Molecular Genetics, VIB, Universiteitsplein 1, 2610, Antwerp 09/24/2010 4.06 Journal of neurochemistry
Increased caspase activation and decreased TDP-43 solubility in progranulin knockout cortical cultures. Abstract

Haass, Christian

 Ludwig-Maximilians-University and German Center for Neurodegenerative Diseases (DZNE) Munich 06/09/2009 4.06 Journal of neurochemistry
Proteolytic processing of TAR DNA binding protein-43 by caspases produces C-terminal fragments with disease defining properties independent of progranulin. Abstract

Strong, Michael J

 Robarts Research Institute 11/19/2008 4.06 Journal of neurochemistry
Tau phosphorylation at threonine-175 leads to fibril formation and enhanced cell death: implications for amyotrophic lateral sclerosis with cognitive impairment. Abstract

Gitler, Aaron D

 Stanford University School of Medicine 11/27/2010 4.01 Methods (San Diego, Calif.)
TDP-43 toxicity in yeast. Abstract

Mackenzie, Ian R A

 University Hospital of Zürich 09/21/2009 4.00 Brain pathology (Zurich, Switzerland)
FUS-immunoreactive intranuclear inclusions in neurodegenerative disease. Abstract

Bosco, Daryl A

 University of Massachusetts Medical School 07/29/2013 3.87 Journal of cellular physiology
FUS/TLS assembles into stress granules and is a prosurvival factor during hyperosmolar stress. Abstract

Landers, John E

 University of Massachusetts Medical School 11/12/2010 3.81 CNS & neurological disorders drug targets
Genetic determinants of amyotrophic lateral sclerosis as therapeutic targets. Abstract

Trojanowski, John Q

 University of Pennsylvania School of Medicine 10/29/2013 3.81 The Journal of comparative neurology
Comparative survey of the topographical distribution of signature molecular lesions in major neurodegenerative diseases. Abstract

Mann, David M A

 University of Manchester 09/26/2014 3.80 Neuropathology and applied neurobiology
Patterns of microglial cell activation in frontotemporal lobar degeneration. Abstract

Revesz, Tamas

 University College London 04/22/2014 3.80 Neuropathology and applied neurobiology
A pathogenic progranulin mutation and C9orf72 repeat expansion in a family with frontotemporal dementia. Abstract

Revesz, T

 01/21/2014 3.80 Neuropathology and applied neurobiology
Characteristics of progressive supranuclear palsy presenting with corticobasal syndrome: a cortical variant. Abstract

Shaw, C E

 King's College London 07/10/2013 3.80 Neuropathology and applied neurobiology
Transportin 1 colocalization with Fused in Sarcoma (FUS) inclusions is not characteristic for amyotrophic lateral sclerosis-FUS confirming disrupted nuclear import of mutant FUS and distinguishing it from frontotemporal lobar degeneration with FUS inclusions. Abstract

Shaw, P J

 University of Sheffield 07/10/2013 3.80 Neuropathology and applied neurobiology
Lack of unique neuropathology in amyotrophic lateral sclerosis associated with p.K54E angiogenin (ANG) mutation. Abstract

Cairns, N J

 Aston University 04/26/2013 3.80 Neuropathology and applied neurobiology
Laminar distribution of the pathological changes in sporadic frontotemporal lobar degeneration with transactive response (TAR) DNA-binding protein of 43 kDa (TDP-43) proteinopathy: a quantitative study using polynomial curve fitting. Abstract

Wakabayashi, K

 Hirosaki University Graduate School of Medicine 05/11/2012 3.80 Neuropathology and applied neurobiology
FUS immunoreactivity of neuronal and glial intranuclear inclusions in intranuclear inclusion body disease. Abstract

Mann, David M A

 University of Manchester 04/13/2012 3.80 Neuropathology and applied neurobiology
Nuclear Carrier and RNA Binding Proteins in Frontotemporal Lobar Degeneration associated with Fused in Sarcoma (FUS) pathological changes. Abstract

Takahashi, H

 University of Niigata 01/11/2012 3.80 Neuropathology and applied neurobiology
Co-occurrence of argyrophilic grain disease in sporadic amyotrophic lateral sclerosis. Abstract

Cairns, N J

 Aston University 01/09/2012 3.80 Neuropathology and applied neurobiology
A quantitative study of the neuropathology of 32 sporadic and familial cases of frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP). Abstract

Trojanowski, J Q

 University of Pennsylvania School of Medicine 06/23/2011 3.80 Neuropathology and applied neurobiology
TDP-43 pathology occurs infrequently in multiple system atrophy. Abstract

Mann, D M A

 Newcastle University 06/23/2011 3.80 Neuropathology and applied neurobiology
Synaptic changes in frontotemporal lobar degeneration: correlation with MAPT haplotype and APOE genotype. Abstract

Wakabayashi, K

 Hirosaki University Graduate School of Medicine 03/15/2010 3.80 Neuropathology and applied neurobiology
Relationship between Bunina bodies and TDP-43 inclusions in spinal anterior horn in amyotrophic lateral sclerosis. Abstract

Petrucelli, L

 Mayo Clinic 02/19/2010 3.80 Neuropathology and applied neurobiology
Review: transactive response DNA-binding protein 43 (TDP-43): mechanisms of neurodegeneration. Abstract

Wszolek, Zbigniew K

 Mayo Clinic 08/23/2008 3.80 Parkinsonism & related disorders
Pallidonigral TDP-43 pathology in Perry syndrome. Abstract

Josephs, Keith A

 Mayo Clinic 03/06/2008 3.80 Parkinsonism & related disorders
Photophobia, visual hallucinations, and REM sleep behavior disorder in progressive supranuclear palsy and corticobasal degeneration: a prospective study. Abstract

Baloh, Robert H

 Washington University School of Medicine 08/24/2011 3.79 The FEBS journal
TDP-43: the relationship between protein aggregation and neurodegeneration in amyotrophic lateral sclerosis and frontotemporal lobar degeneration. Abstract

Baralle, Francisco E

 International Centre for Genetic Engineering and Biotechnology (ICGEB), 34012 Trieste 08/24/2011 3.79 The FEBS journal
TDP-43: new aspects of autoregulation mechanisms in RNA binding proteins and their connection with human disease. Abstract

Landers, J E

 University of Massachusetts Medical School 01/13/2011 3.71 American journal of medical genetics. Part B, Neuropsychiatric genetics : the official publication of the International Society of Psychiatric Genetics
Mutational analysis reveals the FUS homolog TAF15 as a candidate gene for familial amyotrophic lateral sclerosis. Abstract

Josephs, K A

 Mayo Clinic 06/07/2013 3.69 European journal of neurology : the official journal of the European Federation of Neurological Societies
Midbrain atrophy is not a biomarker of progressive supranuclear palsy pathology. Abstract

Dickson, D W

 Mayo Clinic 03/03/2010 3.69 European journal of neurology : the official journal of the European Federation of Neurological Societies
Caudate atrophy on MRI is a characteristic feature of FTLD-FUS. Abstract

Robberecht, W

 University Hospital Gasthuisberg 11/13/2009 3.69 European journal of neurology : the official journal of the European Federation of Neurological Societies
The occurrence of mutations in FUS in a Belgian cohort of patients with familial ALS. Abstract

Wszolek, Z K

 Mayo Clinic 03/31/2009 3.69 European journal of neurology : the official journal of the European Federation of Neurological Societies
GRN 3'UTR+78 C>T is not associated with risk for Parkinson's disease. Abstract

Haass, Christian

 Ludwig-Maximilians-University and German Center for Neurodegenerative Diseases (DZNE) Munich 04/02/2013 3.66 Molecular and cellular neurosciences
Fused in sarcoma (FUS): an oncogene goes awry in neurodegeneration. Abstract

Petrucelli, Leonard

 Mayo Clinic 12/29/2012 3.66 Molecular and cellular neurosciences
RNA-mediated toxicity in neurodegenerative disease. Abstract

Klein, Ronald L

 Louisiana State University Health Sciences Center 07/21/2011 3.63 The European journal of neuroscience
Genetic strategies to study TDP-43 in rodents and to develop preclinical therapeutics for amyotrophic lateral sclerosis. Abstract

Lee, Virginia M Y

 University of Pennsylvania School of Medicine 02/05/2013 3.54 FEBS letters
Neurofibrillary tangle-like tau pathology induced by synthetic tau fibrils in primary neurons over-expressing mutant tau. Abstract

Baralle, Francisco E

 International Centre for Genetic Engineering and Biotechnology (ICGEB), 34012 Trieste 04/19/2009 3.54 FEBS letters
Depletion of TDP-43 affects Drosophila motoneurons terminal synapsis and locomotive behavior. Abstract

Van Broeckhoven, Christine

 Neurodegenerative Brain Diseases Group, Department of Molecular Genetics, VIB, Universiteitsplein 1, B-2610 Antwerpen 03/16/2012 3.52 Annals of medicine
The molecular basis of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum. Abstract

Sobue, Gen

 Nagoya University Graduate School of Medicine 10/02/2013 3.47 Journal of neurology
Amyotrophic lateral sclerosis: an update on recent genetic insights. Abstract

Wakabayashi, Koichi

 Hirosaki University Graduate School of Medicine 02/23/2010 3.47 Journal of neurology
Progressive anterior operculum syndrome due to FTLD-TDP: a clinico-pathological investigation. Abstract

Trojanowski, John Q

 University of Pennsylvania School of Medicine 03/07/2009 3.47 Journal of neurology
Amyotrophic lateral sclerosis, frontotemporal dementia and beyond: the TDP-43 diseases. Abstract

Hardiman, Orla

 Trinity College Dublin 02/17/2009 3.47 Journal of neurology
The management of amyotrophic lateral sclerosis. Abstract

Grossman, Murray

 University of Pennsylvania School of Medicine 08/11/2009 3.46 Current neurology and neuroscience reports
TDP-43 and frontotemporal dementia. Abstract

Rouleau, Guy A

 Centre of Excellence in Neurosciences of Université de Montréal, CHUM Research Center, Montreal, Québec 04/07/2009 3.46 Current neurology and neuroscience reports
Recent advances in the genetics of amyotrophic lateral sclerosis. Abstract

Julien, J-P

 Laval University 01/03/2012 3.38 Neuroscience
Methylene blue administration fails to confer neuroprotection in two amyotrophic lateral sclerosis mouse models. Abstract

Landers, John E

 University of Massachusetts Medical School 03/27/2010 3.35 Neurogenetics
RNA processing pathways in amyotrophic lateral sclerosis. Abstract

Shaw, Pamela J

 University of Sheffield 09/17/2009 3.35 Neurogenetics
Broad clinical phenotypes associated with TAR-DNA binding protein (TARDBP) mutations in amyotrophic lateral sclerosis. Abstract

Julien, Jean-Pierre

 Department of Psychiatry and Neuroscience of Laval University 08/20/2010 3.25 Progress in neuro-psychopharmacology & biological psychiatry
ALS pathogenesis: recent insights from genetics and mouse models. Abstract

Baralle, Francisco E

 International Centre for Genetic Engineering and Biotechnology (ICGEB), 34012 Trieste 11/22/2013 3.24 Biochemical Society transactions
The role of TDP-43 in the pathogenesis of ALS and FTLD. Abstract

Van Broeckhoven, Christine

 Neurodegenerative Brain Diseases Group, Department of Molecular Genetics, VIB, Universiteitsplein 1, B-2610 Antwerpen 07/26/2011 3.24 Biochemical Society transactions
The role of mutant TAR DNA-binding protein 43 in amyotrophic lateral sclerosis and frontotemporal lobar degeneration. Abstract

Blair, Ian P

 ANZAC Research Institute 03/16/2012 3.09 Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases
A novel TARDBP insertion/deletion mutation in the flail arm variant of amyotrophic lateral sclerosis. Abstract

Landers, John E

 University of Massachusetts Medical School 03/13/2012 3.09 Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases
Prevalence of Huntington's disease gene CAG repeat alleles in sporadic amyotrophic lateral sclerosis patients. Abstract

Strong, Michael J

 Robarts Research Institute 01/03/2012 3.09 Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases
Widespread neuronal and glial hyperphosphorylated tau deposition in ALS with cognitive impairment. Abstract

Rouleau, Guy A

 Centre of Excellence in Neurosciences of Université de Montréal, CHUM Research Center, Montreal, Québec 01/24/2011 3.09 Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases
Identification of novel FUS mutations in sporadic cases of amyotrophic lateral sclerosis. Abstract

van Den Berg, Leonard H

 University Medical Center Utrecht 09/03/2010 3.09 Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases
No evidence of microbleeds in ALS patients at 7 Tesla MRI. Abstract

Wszolek, Zbigniew K

 Mayo Clinic 09/24/2013 3.06 Neuro-degenerative diseases
Familial progressive supranuclear palsy: a literature review. Abstract

Baloh, Robert H

 Washington University School of Medicine 12/03/2010 3.06 Neuro-degenerative diseases
TDP-43-based animal models of neurodegeneration: new insights into ALS pathology and pathophysiology. Abstract

Rademakers, Rosa

 Mayo Clinic 03/03/2010 3.06 Neuro-degenerative diseases
Common variant in GRN is a genetic risk factor for hippocampal sclerosis in the elderly. Abstract

Josephs, Keith A

 Mayo Clinic Rochester 03/19/2009 3.06 Neuro-degenerative diseases
MRI correlates of protein deposition and disease severity in postmortem frontotemporal lobar degeneration. Abstract

Shaw, Pamela J

 University of Sheffield 09/23/2011 3.04 BMC neuroscience
A comparison of in vitro properties of resting SOD1 transgenic microglia reveals evidence of reduced neuroprotective function. Abstract

Strong, Michael J

 Robarts Research Institute 03/16/2009 2.96 Journal of neuroimmunology
The complement factor C5a receptor is upregulated in NFL-/- mouse motor neurons. Abstract

Baloh, Robert H

 Washington University School of Medicine 01/01/2011 2.85 Prion
Implications of the prion-related Q/N domains in TDP-43 and FUS. Abstract

Wakabayashi, Koichi

 Hirosaki University Graduate School of Medicine 06/05/2012 2.74 Journal of neuroscience research
p62/sequestosome 1 binds to TDP-43 in brains with frontotemporal lobar degeneration with TDP-43 inclusions. Abstract

Cairns, Nigel J

 Aston University 06/08/2012 2.73 Journal of neural transmission (Vienna, Austria : 1996)
Different molecular pathologies result in similar spatial patterns of cellular inclusions in neurodegenerative disease: a comparative study of eight disorders. Abstract

Cairns, Nigel J

 Aston University 07/27/2011 2.73 Journal of neural transmission (Vienna, Austria : 1996)
Spatial patterns of FUS-immunoreactive neuronal cytoplasmic inclusions (NCI) in neuronal intermediate filament inclusion disease (NIFID). Abstract

Cairns, Nigel J

 Aston University 12/10/2009 2.73 Journal of neural transmission (Vienna, Austria : 1996)
Neuropathological heterogeneity in frontotemporal lobar degeneration with TDP-43 proteinopathy: a quantitative study of 94 cases using principal components analysis. Abstract

Kwak, Shin

 The University of Tokyo 12/16/2013 2.73 Brain research
The molecular link between inefficient GluA2 Q/R site-RNA editing and TDP-43 pathology in motor neurons of sporadic amyotrophic lateral sclerosis patients. Abstract

Baralle, Francisco E

 International Centre for Genetic Engineering and Biotechnology (ICGEB), 34012 Trieste 02/22/2012 2.73 Brain research
Role of selected mutations in the Q/N rich region of TDP-43 in EGFP-12xQ/N-induced aggregate formation. Abstract

Petrucelli, Leonard

 Mayo Clinic 01/28/2012 2.73 Brain research
Progranulin: an emerging target for FTLD therapies. Abstract

Neumann, Manuela

 University of British Columbia and Vancouver General Hospital 12/13/2011 2.73 Brain research
FET proteins in frontotemporal dementia and amyotrophic lateral sclerosis. Abstract

Strong, Michael J

 Robarts Research Institute 10/06/2009 2.73 Brain research
Tar DNA binding protein of 43 kDa (TDP-43), 14-3-3 proteins and copper/zinc superoxide dismutase (SOD1) interact to modulate NFL mRNA stability. Implications for altered RNA processing in amyotrophic lateral sclerosis (ALS). Abstract

Strong, Michael J

 Robarts Research Institute 07/18/2009 2.73 Brain research
Cytosolic TDP-43 expression following axotomy is associated with caspase 3 activation in NFL-/- mice: support for a role for TDP-43 in the physiological response to neuronal injury. Abstract

Strong, Michael J

 Robarts Research Institute 11/01/2008 2.73 Brain research
Divergent patterns of cytosolic TDP-43 and neuronal progranulin expression following axotomy: implications for TDP-43 in the physiological response to neuronal injury. Abstract

Xia, Xu-Gang

 Thomas Jefferson University 03/04/2011 2.70 International journal of biological sciences
TDP-43 potentiates alpha-synuclein toxicity to dopaminergic neurons in transgenic mice. Abstract

Van Broeckhoven, Christine

 University of Antwerp-CDE 04/13/2010 2.63 Biomarkers in medicine
Role of progranulin as a biomarker for Alzheimer's disease. Abstract

Sobue, Gen

 Nagoya University Graduate School of Medicine 01/23/2012 2.60 International journal of molecular sciences
Disruption of axonal transport in motor neuron diseases. Abstract

Neumann, Manuela

 University of British Columbia and Vancouver General Hospital 01/09/2009 2.60 International journal of molecular sciences
Molecular neuropathology of TDP-43 proteinopathies. Abstract

Rouleau, Guy A

 Centre of Excellence in Neurosciences of Université de Montréal, CHUM Research Center, Montreal, Québec 12/16/2010 2.57 Journal of human genetics
Identification of a FUS splicing mutation in a large family with amyotrophic lateral sclerosis. Abstract

Siddique, Teepu

 Northwestern University 09/07/2011 2.50 Journal of molecular neuroscience : MN
Making connections: pathology and genetics link amyotrophic lateral sclerosis with frontotemporal lobe dementia. Abstract

Kumar-Singh, Samir

 Neurodegenerative Brain Diseases Group, Department of Molecular Genetics, VIB, Universiteitsplein 1, 2610, Antwerp 08/24/2011 2.50 Journal of molecular neuroscience : MN
Progranulin and TDP-43: mechanistic links and future directions. Abstract

Petrucelli, Leonard

 Mayo Clinic 08/03/2011 2.50 Journal of molecular neuroscience : MN
Rodent models of TDP-43 proteinopathy: investigating the mechanisms of TDP-43-mediated neurodegeneration. Abstract

Grossman, Murray

 University of Pennsylvania School of Medicine 07/22/2011 2.50 Journal of molecular neuroscience : MN
Biomarkers to identify the pathological basis for frontotemporal lobar degeneration. Abstract

Josephs, Keith A

 Mayo Clinic 07/01/2011 2.50 Journal of molecular neuroscience : MN
Neuropathology of frontotemporal lobar degeneration-tau (FTLD-tau). Abstract

Akiyama, Haruhiko

 Tokyo Institute of Psychiatry 06/16/2011 2.50 Journal of molecular neuroscience : MN
Molecular dissection of TDP-43 proteinopathies. Abstract

Gitler, Aaron D

 Stanford University School of Medicine 06/10/2011 2.50 Journal of molecular neuroscience : MN
Model organisms reveal insight into human neurodegenerative disease: ataxin-2 intermediate-length polyglutamine expansions are a risk factor for ALS. Abstract

Rademakers, Rosa

 Mayo Clinic 05/28/2011 2.50 Journal of molecular neuroscience : MN
Human genetics as a tool to identify progranulin regulators. Abstract

Van Broeckhoven, Christine

 Neurodegenerative Brain Diseases Group, Department of Molecular Genetics, VIB, Universiteitsplein 1, B-2610 Antwerpen 05/26/2011 2.50 Journal of molecular neuroscience : MN
TMEM106B a novel risk factor for frontotemporal lobar degeneration. Abstract

Isaacs, Adrian M

 UCL Institute of Neurology 05/21/2011 2.50 Journal of molecular neuroscience : MN
Novel types of frontotemporal lobar degeneration: beyond tau and TDP-43. Abstract

Josephs, Keith A

 Mayo Clinic 05/10/2011 2.50 Journal of molecular neuroscience : MN
Imaging signatures of molecular pathology in behavioral variant frontotemporal dementia. Abstract

Wakabayashi, Koichi

 Hirosaki University Graduate School of Medicine 11/15/2012 2.48 Biochemical and biophysical research communications
Brain expression level and activity of HDAC6 protein in neurodegenerative dementia. Abstract

Cairns, Nigel J

 Aston University 12/14/2010 2.48 Histology and histopathology
A morphometric study of the spatial patterns of TDP-43 immunoreactive neuronal inclusions in frontotemporal lobar degeneration (FTLD) with progranulin (GRN) mutation. Abstract

Siddique, Teepu

 Northwestern University 01/16/2012 2.37 Muscle & nerve
UBQLN2/P62 cellular recycling pathways in amyotrophic lateral sclerosis and frontotemporal dementia. Abstract

Mackenzie, Ian R A

 University Hospital of Zürich 07/26/2010 2.37 Muscle & nerve
Fus gene mutations in familial and sporadic amyotrophic lateral sclerosis. Abstract

Sobue, Gen

 Nagoya University Graduate School of Medicine 06/08/2011 2.35 Journal of the neurological sciences
Behavioral changes in early ALS correlate with voxel-based morphometry and diffusion tensor imaging. Abstract

Strong, Michael J

 Robarts Research Institute 10/18/2009 2.35 Journal of the neurological sciences
The evidence for altered RNA metabolism in amyotrophic lateral sclerosis (ALS). Abstract

Wszolek, Zbigniew K

 Mayo Clinic 09/04/2009 2.35 Journal of the neurological sciences
Elucidating the genetics and pathology of Perry syndrome. Abstract

Kawakami, Hideshi

 Hiroshima University 05/02/2009 2.35 Journal of the neurological sciences
Screening for TARDBP mutations in Japanese familial amyotrophic lateral sclerosis. Abstract

Baloh, Robert H

 Washington University School of Medicine 11/01/2013 2.34 Neurologic clinics
Clinical neurogenetics: amyotrophic lateral sclerosis. Abstract

Kwak, Shin

 The University of Tokyo 03/10/2012 2.25 Neuroscience research
The abnormal processing of TDP-43 is not an upstream event of reduced ADAR2 activity in ALS motor neurons. Abstract

Kwak, Shin

 University of Tokyo 02/18/2012 2.25 Neuroscience research
RNA editing of the Q/R site of GluA2 in different cultured cell lines that constitutively express different levels of RNA editing enzyme ADAR2. Abstract

Kwak, Shin

 University of Tokyo 03/25/2009 2.25 Neuroscience research
Effects of antidepressants on GluR2 Q/R site-RNA editing in modified HeLa cell line. Abstract

Kawakami, Hideshi

 Hiroshima University 12/26/2012 2.16 Geriatrics & gerontology international
Optineurin and amyotrophic lateral sclerosis. Abstract

Wakabayashi, Koichi

 Hirosaki University Graduate School of Medicine 08/28/2012 2.11 Neuroscience letters
Endosomal sorting related protein CHMP2B is localized in Lewy bodies and glial cytoplasmic inclusions in α-synucleinopathy. Abstract

Wakabayashi, Koichi

 Hirosaki University Graduate School of Medicine 06/19/2012 2.11 Neuroscience letters
Autophagy-related proteins (p62, NBR1 and LC3) in intranuclear inclusions in neurodegenerative diseases. Abstract

Dickson, Dennis W

 Mayo Clinic 01/12/2011 2.11 Neuroscience letters
Disease specificity and pathologic progression of tau pathology in brainstem nuclei of Alzheimer's disease and progressive supranuclear palsy. Abstract

Kwak, Shin

 The University of Tokyo 04/16/2010 2.08 Journal of pharmacological sciences
Novel etiological and therapeutic strategies for neurodiseases: RNA editing enzyme abnormality in sporadic amyotrophic lateral sclerosis. Abstract

Wakabayashi, Koichi

 Hirosaki University Graduate School of Medicine 12/09/2013 2.02 Neuropathology : official journal of the Japanese Society of Neuropathology
Accumulation of the sigma-1 receptor is common to neuronal nuclear inclusions in various neurodegenerative diseases. Abstract

Wakabayashi, Koichi

 Hirosaki University Graduate School of Medicine 07/29/2013 2.02 Neuropathology : official journal of the Japanese Society of Neuropathology
ALS-associated protein FIG4 is localized in Pick and Lewy bodies, and also neuronal nuclear inclusions, in polyglutamine and intranuclear inclusion body diseases. Abstract

Takahashi, Hitoshi

 Nishi-Niigata Chuo National Hospital 07/29/2013 2.02 Neuropathology : official journal of the Japanese Society of Neuropathology
TDP-43 pathology in polyglutamine diseases: with reference to amyotrphic lateral sclerosis. Abstract

Wakabayashi, Koichi

 Hirosaki University Graduate School of Medicine 06/19/2013 2.02 Neuropathology : official journal of the Japanese Society of Neuropathology
Valosin-containing protein immunoreactivity in tauopathies, synucleinopathies, polyglutamine diseases and intranuclear inclusion body disease. Abstract

Wakabayashi, Koichi

 Hirosaki University Graduate School of Medicine 05/27/2013 2.02 Neuropathology : official journal of the Japanese Society of Neuropathology
Co-localization of Bunina bodies and TDP-43 inclusions in lower motor neurons in amyotrophic lateral sclerosis. Abstract

Takahashi, Hitoshi

 Nishi-Niigata Chuo National Hospital 06/04/2012 2.02 Neuropathology : official journal of the Japanese Society of Neuropathology
A fatal neuromuscular disease in an adult patient after poliomyelitis in early childhood: consideration of the pathology of post-polio syndrome. Abstract

Sobue, Gen

 Nagoya University Graduate School of Medicine 12/22/2011 2.02 Neuropathology : official journal of the Japanese Society of Neuropathology
Neuropathology and omics in motor neuron diseases. Abstract

Wakabayashi, Koichi

 Hirosaki University Graduate School of Medicine 11/28/2011 2.02 Neuropathology : official journal of the Japanese Society of Neuropathology
Immunohistochemical analysis of Marinesco bodies, using antibodies against proteins implicated in the ubiquitin-proteasome system, autophagy and aggresome formation. Abstract

Takahashi, Hitoshi

 Nishi-Niigata Chuo National Hospital 11/21/2011 2.02 Neuropathology : official journal of the Japanese Society of Neuropathology
Primary lateral sclerosis: upper-motor-predominant amyotrophic lateral sclerosis with frontotemporal lobar degeneration--immunohistochemical and biochemical analyses of TDP-43. Abstract

Akiyama, Haruhiko

 Tokyo Institute of Psychiatry 10/06/2011 2.02 Neuropathology : official journal of the Japanese Society of Neuropathology
Progressive nonfluent aphasia: a rare clinical subtype of FTLD-TDP in Japan. Abstract

Wakabayashi, Koichi

 Hirosaki University Graduate School of Medicine 02/01/2011 2.02 Neuropathology : official journal of the Japanese Society of Neuropathology
Accumulation of histone deacetylase 6, an aggresome-related protein, is specific to Lewy bodies and glial cytoplasmic inclusions. Abstract

Wakabayashi, Koichi

 Hirosaki University Graduate School of Medicine 12/22/2010 2.02 Neuropathology : official journal of the Japanese Society of Neuropathology
Enhancement of native and phosphorylated TDP-43 immunoreactivity by proteinase K treatment following autoclave heating. Abstract

Wakabayashi, Koichi

 Hirosaki University Graduate School of Medicine 11/25/2010 2.02 Neuropathology : official journal of the Japanese Society of Neuropathology
Widespread occurrence of eosinophilic neuronal cytoplasmic inclusions in an asymptomatic adult: a novel ubiquitin-negative filamentous inclusion. Abstract

Trojanowski, John Q

 University of Pennsylvania School of Medicine 01/25/2010 2.02 Neuropathology : official journal of the Japanese Society of Neuropathology
Amyotrophic lateral sclerosis and frontotemporal lobar degeneration: a spectrum of TDP-43 proteinopathies. Abstract

Akiyama, Haruhiko

 Tokyo Institute of Psychiatry 01/19/2010 2.02 Neuropathology : official journal of the Japanese Society of Neuropathology
Phosphorylated and cleaved TDP-43 in ALS, FTLD and other neurodegenerative disorders and in cellular models of TDP-43 proteinopathy. Abstract

Wakabayashi, Koichi

 Hirosaki University Graduate School of Medicine 08/23/2009 2.02 Neuropathology : official journal of the Japanese Society of Neuropathology
Sporadic amyotrophic lateral sclerosis with pallido-nigro-luysian degeneration: a TDP-43 immunohistochemical study. Abstract

Takahashi, Hitoshi

 Nishi-Niigata Chuo National Hospital 01/02/2009 2.02 Neuropathology : official journal of the Japanese Society of Neuropathology
Sporadic amyotrophic lateral sclerosis: Widespread multisystem degeneration with TDP-43 pathology in a patient after long-term survival on a respirator. Abstract
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