LAB
INDEX
Showing search results for "Intranuclear Inclusions"
Impact
Papers
Jobs
Conferences
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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