Cited by 195Amyotrophic Lateral Sclerosis ResearchAlzheimer's disease research and treatmentsAmyloidosis: Diagnosis, Treatment, Outcomes

The DOI matches its Crossref record; 106 of the 108 references it cites were found in a registry or an index.

Amyotrophic lateral sclerosis and frontotemporal lobar degeneration: A spectrum of TDP-43 proteinopathies

Open access

Geser, Felix, Lee, Virginia M.-Y., Trojanowski, John Q.·2010·Neuropathology

DOI 10.1111/j.1440-1789.2009.01091.xPMC PMC3131978

Open this paper in CoChat — free account

Abstract

It is now established that pathological transactive response DNA-binding protein with a Mr of 43 kD (TDP-43) on sodium dodecyl sulfate-polyacrylamide gel electrophoresis is the major disease protein in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) with ubiquitin-positive inclusions (now known as FTLD-TDP). In fact, the discovery of pathological TDP-43 solidified the idea that these disorders are multi-system diseases and this led to the concept of a TDP-43 proteinopathy as a spectrum of disorders comprised of different clinical and pathological entities extending from ALS to ALS with cognitive impairment/dementia and FTLD-TDP without or with motor neuron disease (FTLD-MND). These align along a broad disease continuum sharing similar pathogenetic mechanisms linked to pathological TDP-43. We here review salient findings in the development of a concept of TDP-43 proteinopathy as a novel group of neurodegenerative diseases similar in concept to alpha-synucleinopathies and tauopathies.

The claims and statistics of this paper haven’t been checked yet.

Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.

Integrity signals

Registry

Crossref · 10.1111/j.1440-1789.2009.01091.x

Cites

106 of 106 references verified0 citation errors · 0 not checkable

Statistics

Claims the paper makes

The claims and statistics of this paper haven’t been checked yet.

Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.

Claims other papers make about this one

Each is checked against this paper’s own text: does it say what the citing paper says it does?

Self-check

Percentages, sums and reported differences re-computed from the paper’s own numbers.

The self-check needs the paper’s full text.

Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis
Neumann, M., Sampathu, D. M., Kwong, L. K., Truax, A. C., Micsenyi, M. C., Chou, T. T., Bruce, J., Schuck, T., Grossman, M., Clark, C. M., et al. (2006) · Science · doi:10.1126/science.1134108 · Cited by 6,576
Self-citation
El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis
Brooks, B. R., Miller, R. G., Swash, M., & Munsat, T. L. (2000) · Amyotrophic Lateral Sclerosis and Other Motor Neuron Disorders · doi:10.1080/146608200300079536 · Cited by 5,433
Frontotemporal lobar degeneration: A consensus on clinical diagnostic criteria
Neary, D., Snowden, J. S., Gustafson, L., Passant, U., Stuss, D., Black, S., Freedman, M., Kertesz, A., Robert, P. H., Albert, M., et al. (1998) · Neurology · doi:10.1212/wnl.51.6.1546 · Cited by 5,047
Primary Progressive Aphasia
Weıntraub, S. (1990) · Archives of Neurology · doi:10.1001/archneur.1990.00530120075013 · Cited by 3,018
The Microprocessor complex mediates the genesis of microRNAs
Gregory, R. I., Yan, K.-P., Amuthan, G., Chendrimada, T. P., Doratotaj, B., Cooch, N. S., & Shiekhattar, R. (2004) · Nature · doi:10.1038/nature03120 · Cited by 2,852
TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
Arai, T., Hasegawa, M., Akiyama, H., Ikeda, K., Nonaka, T., Mori, H., Mann, D., Tsuchiya, K., Yoshida, M., Hashizume, Y., et al. (2006) · Biochemical and Biophysical Research Communications · doi:10.1016/j.bbrc.2006.10.093 · Cited by 2,732
TDP-43 Mutations in Familial and Sporadic Amyotrophic Lateral Sclerosis
Sreedharan, J., Blair, I. P., Tripathi, V. B., Hu, X., Vance, C., Rogelj, B., Ackerley, S., Durnall, J. C., Williams, K. L., Buratti, E., et al. (2008) · Science · doi:10.1126/science.1154584 · Cited by 2,630
Mutations in FUS, an RNA Processing Protein, Cause Familial Amyotrophic Lateral Sclerosis Type 6
Vance, C. A., Rogelj, B., Hortobágyi, T., Vos, K. J. D., Nishimura, A. L., Sreedharan, J., Hu, X., Smith, B. N., Ruddy, D., Wright, P. D., et al. (2009) · Science · doi:10.1126/science.1165942 · Cited by 2,625
Mutations in the FUS/TLS Gene on Chromosome 16 Cause Familial Amyotrophic Lateral Sclerosis
Kwiatkowski, T. J., Bosco, D. A., LeClerc, A. L., Tamrazian, E., Vanderburg, C. R., Russ, C., Davis, A., Gilchrist, J. M., Kasarskis, E. J., Munsat, T. L., et al. (2009) · Science · doi:10.1126/science.1166066 · Cited by 2,624
El escorial World Federation of Neurology criteria for the diagnosis of amyotrophic lateral sclerosis
Brooks, B. R. (1994) · Journal of the Neurological Sciences · doi:10.1016/0022-510x(94)90191-0 · Cited by 2,171
Mutations in progranulin cause tau-negative frontotemporal dementia linked to chromosome 17
Baker, M., Mackenzie, I. R., Pickering-Brown, S. M., Gass, J., Rademakers, R., Lindholm, C., Snowden, J., Adamson, J., Sadovnick, A. D., Rollinson, S., et al. (2006) · Nature · doi:10.1038/nature05016 · Cited by 2,058
TARDBP mutations in individuals with sporadic and familial amyotrophic lateral sclerosis
Kabashi, E., Valdmanis, P. N., Dion, P., Spiegelman, D., McConkey, B. J., Velde, C. V., Bouchard, J.-P., Lacomblez, L., Pochigaeva, K., Salachas, F., et al. (2008) · Nature Genetics · doi:10.1038/ng.132 · Cited by 1,544
Null mutations in progranulin cause ubiquitin-positive frontotemporal dementia linked to chromosome 17q21
Cruts, M., Gijselinck, I., van der Zee, J., Engelborghs, S., Wils, H., Pirici, D., Rademakers, R., Vandenberghe, R., Dermaut, B., Martin, J.-J., et al. (2006) · Nature · doi:10.1038/nature05017 · Cited by 1,512
Clinical and Pathological Diagnosis of Frontotemporal Dementia
McKhann, G. M. (2001) · Archives of Neurology · doi:10.1001/archneur.58.11.1803 · Cited by 1,478
Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the Consortium for Frontotemporal Lobar Degeneration
Cairns, N. J., Bigio, E. H., Mackenzie, I. R. A., Neumann, M., Lee, V. M.-Y., Hatanpaa, K. J., White, C. L., Schneider, J. A., Grinberg, L. T., Halliday, G., et al. (2007) · Acta Neuropathologica · doi:10.1007/s00401-007-0237-2 · Cited by 1,110
Self-citation
DNA/RNA Helicase Gene Mutations in a Form of Juvenile Amyotrophic Lateral Sclerosis (ALS4)
Chen, Y.-Z., Bennett, C. L., Huynh, H. M., Blair, I. P., Puls, I., Irobi, J., Dierick, I., Abel, A., Kennerson, M. L., Rabin, B. A., et al. (2004) · The American Journal of Human Genetics · doi:10.1086/421054 · Cited by 809
Phosphorylated TDP‐43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis
Hasegawa, M., Arai, T., Nonaka, T., Kametani, F., Yoshida, M., Hashizume, Y., Beach, T. G., Buratti, E., Baralle, F., Morita, M., et al. (2008) · Annals of Neurology · doi:10.1002/ana.21425 · Cited by 777
Cloning and characterization of a novel cellular protein, TDP-43, that binds to human immunodeficiency virus type 1 TAR DNA sequence motifs
Ou, S.-H. I., Wu, F. K., Harrich, D., Garcı́a-Martı́nez, L. F., & Gaynor, R. B. (1995) · Journal of Virology · doi:10.1128/jvi.69.6.3584-3596.1995 · Cited by 762
TARDBP mutations in amyotrophic lateral sclerosis with TDP-43 neuropathology: a genetic and histopathological analysis
Van Deerlin, V. M., Leverenz, J. B., Bekris, L. M., Bird, T. D., Yuan, W., Elman, L. B., Clay, D., Wood, E. M., Chen-Plotkin, A. S., Martinez-Lage, M., et al. (2008) · The Lancet Neurology · doi:10.1016/s1474-4422(08)70071-1 · Cited by 723
Self-citation
Structural determinants of the cellular localization and shuttling of TDP-43
Ayala, Y. M., Zago, P., D’Ambrogio, A., Xu, Y.-F., Petrucelli, L., Buratti, E., & Baralle, F. E. (2008) · Journal of Cell Science · doi:10.1242/jcs.038950 · Cited by 643
Nuclear factor TDP‐43 and SR proteins promote in vitro and in vivo CFTR exon 9 skipping
Buratti, E., Dörk, T., Zuccato, E., Pagani, F., Romano, M., & Baralle, F. E. (2001) · The EMBO Journal · doi:10.1093/emboj/20.7.1774 · Cited by 633
TDP‐43 A315T mutation in familial motor neuron disease
Gitcho, M. A., Baloh, R. H., Chakraverty, S., Mayo, K., Norton, J. B., Levitch, D., Hatanpaa, K. J., White, C. L., Bigio, E. H., Caselli, R. J., et al. (2008) · Annals of Neurology · doi:10.1002/ana.21344 · Cited by 633
The evolution and pathology of frontotemporal dementia
Kertesz, A., McMonagle, P., Blair, M., Davidson, W., & Munoz, D. G. (2005) · Brain · doi:10.1093/brain/awh598 · Cited by 609
Phosphorylation of S409/410 of TDP-43 is a consistent feature in all sporadic and familial forms of TDP-43 proteinopathies
Neumann, M., Kwong, L. K., Lee, E. B., Kremmer, E., Flatley, A., Xu, Y., Forman, M. S., Troost, D., Kretzschmar, H. A., Trojanowski, J. Q., et al. (2009) · Acta Neuropathologica · doi:10.1007/s00401-008-0477-9 · Cited by 608
Self-citation
Rethinking ALS: The FUS about TDP-43
Lagier‐Tourenne, C., & Cleveland, D. W. (2009) · Cell · doi:10.1016/j.cell.2009.03.006 · Cited by 596
Frontotemporal dementia
Neary, D., Snowden, J. S., & Mann, D. M. A. (2005) · The Lancet Neurology · doi:10.1016/s1474-4422(05)70223-4 · Cited by 558
Consensus criteria for the diagnosis of frontotemporal cognitive and behavioural syndromes in amyotrophic lateral sclerosis
Strong, M. J., Grace, G. M., Freedman, M., Lomen-Hoerth, C., Woolley, S. C., Goldstein, L. H., Murphy, J. M., Shoesmith, C. L., Rosenfeld, J., Leigh, P. N., et al. (2009) · Amyotrophic Lateral Sclerosis · doi:10.1080/17482960802654364 · Cited by 557
TDP-43 in Familial and Sporadic Frontotemporal Lobar Degeneration with Ubiquitin Inclusions
Cairns, N. J., Neumann, M., Bigio, E. H., Holm, I. E., Troost, D., Hatanpaa, K. J., Foong, C., White, C. L., Schneider, J. A., Kretzschmar, H. A., et al. (2007) · American Journal Of Pathology · doi:10.2353/ajpath.2007.070182 · Cited by 545
Self-citation
Multiple roles of TDP-43 in gene expression, splicing regulation, and human disease
Buratti, E. (2007) · Frontiers in bioscience · doi:10.2741/2727 · Cited by 493
The central nervous system in motor neurone disease
Brownell, B., Oppenheimer, D. R., & Hughes, J. T. (1970) · Journal of Neurology, Neurosurgery & Psychiatry · doi:10.1136/jnnp.33.3.338 · Cited by 483
Frontotemporal dementia: Clinicopathological correlations
Forman, M. S., Farmer, J., Johnson, J. K., Clark, C. M., Arnold, S. E., Coslett, H. B., Chatterjee, A., Hurtig, H. I., Karlawish, J. H., Rosen, H. J., et al. (2006) · Annals of Neurology · doi:10.1002/ana.20873 · Cited by 478
Self-citation
Novel Mutations in TARDBP (TDP-43) in Patients with Familial Amyotrophic Lateral Sclerosis
Rutherford, N. J., Zhang, Y., Baker, M. C., Gass, J. M., Finch, N. A., Xu, Y., Stewart, H. G., Kelley, B. J., Kuntz, K., Crook, R., et al. (2008) · PLoS Genetics · doi:10.1371/journal.pgen.1000193 · Cited by 465
Nomenclature for neuropathologic subtypes of frontotemporal lobar degeneration: consensus recommendations
Mackenzie, I. R., Neumann, M., Bigio, E. H., Cairns, N. J., Alafuzoff, I. I., Kril, J. J., Kovács, G. G., Ghetti, B. F., Halliday, G. M., Holm, I. E., et al. (2008) · Acta Neuropathologica · doi:10.1007/s00401-008-0460-5 · Cited by 425
Self-citation
TDP ‐ 43 mutation in familial amyotrophic lateral sclerosis
Yokoseki, A., Shiga, A., Tan, C., Tagawa, A., Kaneko, H., Koyama, A., Eguchi, H., Tsujino, A., Ikeuchi, T., Kakita, A., et al. (2008) · Annals of Neurology · doi:10.1002/ana.21392 · Cited by 373
TDP43 is a human low molecular weight neurofilament (hNFL) mRNA-binding protein
Strong, M. J., Volkening, K., Hammond, R. R., Yang, W., Strong, W., Leystra‐Lantz, C., & Shoesmith, C. L. (2007) · Molecular and Cellular Neuroscience · doi:10.1016/j.mcn.2007.03.007 · Cited by 362
Enrichment of C-Terminal Fragments in TAR DNA-Binding Protein-43 Cytoplasmic Inclusions in Brain but not in Spinal Cord of Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis
Igaz, L. M., Kwong, L. K., Xu, Y., Truax, A. C., Uryu, K., Neumann, M., Clark, C. M., Elman, L. B., Miller, B. L., Grossman, M., et al. (2008) · American Journal Of Pathology · doi:10.2353/ajpath.2008.080003 · Cited by 332
Self-citation
TARDBP mutations in motoneuron disease with frontotemporal lobar degeneration
Benajiba, L., Ber, I. L., Camuzat, A., Lacoste, M., Thomas-Antérion, C., Couratier, P., Legallic, S., Salachas, F., Hannequin, D., Décousus, M., et al. (2009) · Annals of Neurology · doi:10.1002/ana.21612 · Cited by 322
Heterogeneity of ubiquitin pathology in frontotemporal lobar degeneration: classification and relation to clinical phenotype
Mackenzie, I. R., Baborie, A., Pickering-Brown, S. M., du Plessis, D., Jaros, E., Perry, R. H., Neary, D., Snowden, J. S., & Mann, D. M. A. (2006) · Acta Neuropathologica · doi:10.1007/s00401-006-0138-9 · Cited by 322
Pathological Heterogeneity of Frontotemporal Lobar Degeneration with Ubiquitin-Positive Inclusions Delineated by Ubiquitin Immunohistochemistry and Novel Monoclonal Antibodies
Sampathu, D. M., Neumann, M., Kwong, L. K., Chou, T. T. T., Micsenyi, M. C., Truax, A. C., BRUCE, J. S., Grossman, M., Trojanowski, J. Q., & Lee, V. M. ‐. (2006) · American Journal Of Pathology · doi:10.2353/ajpath.2006.060438 · Cited by 321
Self-citation
Evidence of Multisystem Disorder in Whole-Brain Map of Pathological TDP-43 in Amyotrophic Lateral Sclerosis
Geser, F., Brandmeir, N. J., Kwong, L. K., Martinez‐Lage, M., Elman, L. B., McCluskey, L. F., Xie, S. X., Lee, V. M. ‐., & Trojanowski, J. Q. (2008) · Archives of Neurology · doi:10.1001/archneur.65.5.636 · Cited by 308
Self-citation
Structural diversity and functional implications of the eukaryotic TDP gene family
Wang, H., Wang, I., Bose, J. K., & Shen, C. ‐. J. (2003) · Genomics · doi:10.1016/s0888-7543(03)00214-3 · Cited by 306
Ubiquitinated pathological lesions in frontotemporal lobar degeneration contain the TAR DNA-binding protein, TDP-43
Davidson, Y. S., Kelley, T., Mackenzie, I. R., Pickering-Brown, S. M., du Plessis, D., Neary, D., Snowden, J. S., & Mann, D. M. A. (2007) · Acta Neuropathologica · doi:10.1007/s00401-006-0189-y · Cited by 299
Clinical and Pathological Continuum of Multisystem TDP-43 Proteinopathies
Geser, F., Martinez-Lage, M., Robinson, J., Uryu, K., Neumann, M., Brandmeir, N. J., Xie, S. X., Kwong, L. K., Elman, L., McCluskey, L., et al. (2009) · Archives of Neurology · doi:10.1001/archneurol.2008.558 · Cited by 267
Self-citation
TDP-43 regulates retinoblastoma protein phosphorylation through the repression of cyclin-dependent kinase 6 expression
Ayala, Y. M., Misteli, T., & Baralle, F. E. (2008) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0800546105 · Cited by 235
TDP-43 proteinopathy: the neuropathology underlying major forms of sporadic and familial frontotemporal lobar degeneration and motor neuron disease
Kwong, L. K., Neumann, M., Sampathu, D. M., Lee, V. M. ‐., & Trojanowski, J. Q. (2007) · Acta Neuropathologica · doi:10.1007/s00401-007-0226-5 · Cited by 231
Self-citation
Depletion of TDP 43 overrides the need for exonic and intronic splicing enhancers in the human apoA-II gene
Mercado, P. A. (2005) · Nucleic Acids Research · doi:10.1093/nar/gki897 · Cited by 226
Abnormal phosphorylation of Ser409/410 of TDP‐43 in FTLD‐U and ALS
Inukai, Y., Nonaka, T., Arai, T., Yoshida, M., Hashizume, Y., Beach, T. G., Buratti, E., Baralle, F. E., Akiyama, H., Hisanaga, S., et al. (2008) · FEBS Letters · doi:10.1016/j.febslet.2008.07.027 · Cited by 221
The role of transactive response DNA-binding protein-43 in amyotrophic lateral sclerosis and frontotemporal dementia
Mackenzie, I. R., & Rademakers, R. (2008) · Current Opinion in Neurology · doi:10.1097/wco.0b013e3283168d1d · Cited by 214
High frequency ofTARDBPgene mutations in Italian patients with amyotrophic lateral sclerosis
Corrado, L., Ratti, A., Gellera, C., Buratti, E., Castellotti, B., Carlomagno, Y., Ticozzi, N., Mazzini, L., Testa, L., Taroni, F., et al. (2009) · Human Mutation · doi:10.1002/humu.20950 · Cited by 213
Increased TDP-43 protein in cerebrospinal fluid of patients with amyotrophic lateral sclerosis
Kasai, T., Tokuda, T., Ishigami, N., Sasayama, H., Foulds, P., Mitchell, D. J., Mann, D. M. A., Allsop, D., & Nakagawa, M. (2009) · Acta Neuropathologica · doi:10.1007/s00401-008-0456-1 · Cited by 211
TDP-43 in Cerebrospinal Fluid of Patients With Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis
Steinacker, P., Hendrich, C., Sperfeld, A. D., Jesse, S., von Arnim, C. A. F., Lehnert, S., Pabst, A., Uttner, I., Tumani, H., Lee, V. M.-Y., et al. (2008) · Archives of Neurology · doi:10.1001/archneur.65.11.1481 · Cited by 199
Self-citation
Contribution of TARDBP mutations to sporadic amyotrophic lateral sclerosis
Daoud, H., Valdmanis, P. N., Kabashi, E., Dion, P. A., Dupré, N., Camu, W., Meininger, V., & Rouleau, G. A. (2008) · Journal of Medical Genetics · doi:10.1136/jmg.2008.062463 · Cited by 198
Amyotrophic lateral sclerosis, frontotemporal dementia and beyond: the TDP-43 diseases
Geser, F., Martinez‐Lage, M., Kwong, L. K., Lee, V. M. ‐., & Trojanowski, J. Q. (2009) · Journal of Neurology · doi:10.1007/s00415-009-5069-7 · Cited by 196
Self-citation
TDP-43 Proteinopathy in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis
Neumann, M., Kwong, L. K., Sampathu, D. M., Trojanowski, J. Q., & Lee, V. M. ‐. (2007) · Archives of Neurology · doi:10.1001/archneur.64.10.1388 · Cited by 190
Self-citation
Higher order arrangement of the eukaryotic nuclear bodies
Wang, I., Reddy, N. M., & Shen, C.-K. J. (2002) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.212483099 · Cited by 189
Nuclear Factor TDP-43 Binds to the Polymorphic TG Repeats in CFTR Intron 8 and Causes Skipping of Exon 9: A Functional Link with Disease Penetrance
Buratti, E., Brindisi, A., Pagani, F., & Baralle, F. E. (2004) · The American Journal of Human Genetics · doi:10.1086/420978 · Cited by 187
Sporadic amyotrophic lateral sclerosis: two pathological patterns shown by analysis of distribution of TDP-43-immunoreactive neuronal and glial cytoplasmic inclusions
Nishihira, Y., Tan, C.-F., Onodera, O., Toyoshima, Y., Yamada, M., Morita, T., Nishizawa, M., Kakita, A., & Takahashi, H. (2008) · Acta Neuropathologica · doi:10.1007/s00401-008-0385-z · Cited by 187
Molecular Neuropathology of TDP-43 Proteinopathies
Neumann, M. (2009) · International Journal of Molecular Sciences · doi:10.3390/ijms10010232 · Cited by 172
TDP-43 Overexpression Enhances Exon 7 Inclusion during the Survival of Motor Neuron Pre-mRNA Splicing
Bose, J. K., Wang, I., Li, H., Tarn, W., & Shen, C. ‐. J. (2008) · Journal of Biological Chemistry · doi:10.1074/jbc.m805376200 · Cited by 172
TDP‐43 Redistribution is an Early Event in Sporadic Amyotrophic Lateral Sclerosis
Giordana, M. T., Piccinini, M., Grifoni, S., Marco, G. D., Vercellino, M., Magistrello, M., Pellerino, A., Buccinnà, B., Lupino, E., & RINAUDO, M. T. (2009) · Brain Pathology · doi:10.1111/j.1750-3639.2009.00284.x · Cited by 166
TDP-43 protein in plasma may index TDP-43 brain pathology in Alzheimer’s disease and frontotemporal lobar degeneration
Foulds, P. G., McAuley, E., Gibbons, L., Davidson, Y. S., Pickering-Brown, S. M., Neary, D., Snowden, J. S., Allsop, D., & Mann, D. M. A. (2008) · Acta Neuropathologica · doi:10.1007/s00401-008-0389-8 · Cited by 164
Ubiquitin Immunohistochemistry Suggests Classic Motor Neuron Disease, Motor Neuron Disease With Dementia, and Frontotemporal Dementia of the Motor Neuron Disease Type Represent a Clinicopathologic Spectrum
Mackenzie, I. R., & Feldman, H. (2005) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/01.jnen.0000174335.27708.0a · Cited by 162
Two German Kindreds With Familial Amyotrophic Lateral Sclerosis Due to TARDBP Mutations
Kühnlein, P., Sperfeld, A., Vanmassenhove, B., Deerlin, V. M. V., Lee, V. M. ‐., Trojanowski, J. Q., Kretzschmar, H. A., Ludolph, A. C., & Neumann, M. (2008) · Archives of Neurology · doi:10.1001/archneur.65.9.1185 · Cited by 161
Self-citation
Progranulin genetic variability contributes to amyotrophic lateral sclerosis
Sleegers, K., Brouwers, N., Maurer-Stroh, S., van Es, M. A., Damme, P. V., van Vught, P. W. J., van der Zee, J., Serneels, S., Pooter, T. D., Van den Broeck, M., et al. (2008) · Neurology · doi:10.1212/01.wnl.0000289191.54852.75 · Cited by 161
The syndromes of frontotemporal dysfunction in amyotrophic lateral sclerosis
Strong, M. J. (2008) · Amyotrophic Lateral Sclerosis · doi:10.1080/17482960802372371 · Cited by 154
Overlap between neurodegenerative disorders
Armstrong, R. A., Lantos, P. L., & Cairns, N. J. (2005) · Neuropathology · doi:10.1111/j.1440-1789.2005.00605.x · Cited by 146
TDP-43 in differential diagnosis of motor neuron disorders
Dickson, D. W., Josephs, K. A., & Amador, C. (2007) · Acta Neuropathologica · doi:10.1007/s00401-007-0234-5 · Cited by 143
ALS and FTLD: two faces of TDP‐43 proteinopathy
Liščić, R. M., Grinberg, L. T., Zidar, J., Gitcho, M. A., & Cairns, N. J. (2008) · European Journal of Neurology · doi:10.1111/j.1468-1331.2008.02195.x · Cited by 142
Variations in the progranulin gene affect global gene expression in frontotemporal lobar degeneration
Chen-Plotkin, A. S., Geser, F., Plotkin, J. B., Clark, C. M., Kwong, L. K., Yuan, W., Grossman, M., Van Deerlin, V. M., Trojanowski, J. Q., & Lee, V. M.-Y. (2008) · Human Molecular Genetics · doi:10.1093/hmg/ddn023 · Cited by 135
Self-citation
Ultrastructural localization of TDP-43 in filamentous neuronal inclusions in various neurodegenerative diseases
Lin, W.-L., & Dickson, D. W. (2008) · Acta Neuropathologica · doi:10.1007/s00401-008-0408-9 · Cited by 135
Maturation process of TDP-43-positive neuronal cytoplasmic inclusions in amyotrophic lateral sclerosis with and without dementia
Mori, F., Tanji, K., Zhang, H., Nishihira, Y., Tan, C., Takahashi, H., & Wakabayashi, K. (2008) · Acta Neuropathologica · doi:10.1007/s00401-008-0396-9 · Cited by 135
Evaluation of subcortical pathology and clinical correlations in FTLD-U subtypes
Josephs, K. A., Stroh, A., Dugger, B. N., & Dickson, D. W. (2009) · Acta Neuropathologica · doi:10.1007/s00401-009-0547-7 · Cited by 126
Lack of TDP-43 abnormalities in mutant SOD1 transgenic mice shows disparity with ALS
Robertson, J., Sanelli, T. R., Xiao, S., Yang, W., Horne, P., Hammond, R. R., Pioro, E. P., & Strong, M. J. (2007) · Neuroscience Letters · doi:10.1016/j.neulet.2007.03.066 · Cited by 125
TDP-43: an emerging new player in neurodegenerative diseases
Wang, I., Wu, L., & Shen, C. (2008) · Trends in Molecular Medicine · doi:10.1016/j.molmed.2008.09.001 · Cited by 123
Neuropathology of ALS
Hirano, A. (1996) · Neurology · doi:10.1212/wnl.47.4_suppl_2.63s · Cited by 119
TARDBP (TDP‐43) sequence analysis in patients with familial and sporadic ALS: identification of two novel mutations
Bo, R. D., Ghezzi, S., Corti, S. P., Pandolfo, M., Ranieri, M., Santoro, D., Ghione, I., Prelle, A. C., Orsetti, V., Mancuso, M., et al. (2009) · European Journal of Neurology · doi:10.1111/j.1468-1331.2009.02574.x · Cited by 108
A Novel CpG-free Vertebrate Insulator Silences the Testis-specific SP-10 Gene in Somatic Tissues
Abhyankar, M. M., Urekar, C., & Reddi, P. P. (2007) · Journal of Biological Chemistry · doi:10.1074/jbc.m705811200 · Cited by 108
Chromosome 9p-linked families with frontotemporal dementia associated with motor neuron disease
Ber, I. L., Camuzat, A., Berger, É., Hannequin, D., Laquerrière, A., Golfier, V., Seilhean, D., Viennet, G., Couratier, P., Verpillat, P., et al. (2009) · Neurology · doi:10.1212/wnl.0b013e3181a55f1c · Cited by 98
Clinicopathological characterization of Pick’s disease versus frontotemporal lobar degeneration with ubiquitin/TDP-43-positive inclusions
Yokota, O., Tsuchiya, K., Arai, T., Yagishita, S., Matsubara, O., Mochizuki, A., Tamaoka, A., Kawamura, M., Yoshida, H., Terada, S., et al. (2009) · Acta Neuropathologica · doi:10.1007/s00401-009-0493-4 · Cited by 90
Severe subcortical TDP-43 pathology in sporadic frontotemporal lobar degeneration with motor neuron disease
Brandmeir, N. J., Geser, F., Kwong, L. K., Zimmerman, E. A., Qian, J., Lee, V. M. ‐., & Trojanowski, J. Q. (2007) · Acta Neuropathologica · doi:10.1007/s00401-007-0315-5 · Cited by 87
Self-citation
Neuronal inclusion protein TDP-43 has no primary genetic role in FTD and ALS
Gijselinck, I., Sleegers, K., Engelborghs, S., Robberecht, W., Martin, J., Vandenberghe, R. R., Sciot, R., Dermaut, B., Goossens, D., van der Zee, J., et al. (2008) · Neurobiology of Aging · doi:10.1016/j.neurobiolaging.2007.11.002 · Cited by 85
Expression of ubiquitin-binding protein p62 in ubiquitin-immunoreactive intraneuronal inclusions in amyotrophic lateral sclerosis with dementia: analysis of five autopsy cases with broad clinicopathological spectrum
Nakano, T., Nakaso, K., Ohama, E., & Nakashima, K. (2004) · Acta Neuropathologica · doi:10.1007/s00401-004-0821-7 · Cited by 78
Survival in two variants of tau-negative frontotemporal lobar degeneration: FTLD-U vs FTLD-MND
Josephs, K. A., Knopman, D. S., Whitwell, J. L., Boeve, B. F., Parisi, J. E., Petersen, R. C., & Dickson, D. W. (2005) · Neurology · doi:10.1212/01.wnl.0000173178.67986.7f · Cited by 76
TDP-43 is a culprit in human neurodegeneration, and not just an innocent bystander
Banks, G. T., Kuta, A., Isaacs, A. M., & Fisher, E. (2008) · Mammalian Genome · doi:10.1007/s00335-008-9117-x · Cited by 73
TDP-43 Is Not a Common Cause of Sporadic Amyotrophic Lateral Sclerosis
Guerreiro, R. J., Schymick, J. C., Crews, C. K., Singleton, A., Hardy, J., & Traynor, B. J. (2008) · PLoS ONE · doi:10.1371/journal.pone.0002450 · Cited by 71
Accumulation of TDP-43 and α-actin in an amyotrophic lateral sclerosis patient with the K17I ANG mutation
Seilhean, D., Cazeneuve, C., Thuriès, V., Russaouen, O., Millecamps, S., Salachas, F., Meininger, V., LeGuern, E., & Duyckaerts, C. (2009) · Acta Neuropathologica · doi:10.1007/s00401-009-0545-9 · Cited by 71
Sporadic amyotrophic lateral sclerosis of long duration is associated with relatively mild TDP-43 pathology
Nishihira, Y., Tan, C., Hoshi, Y., Iwanaga, K., Yamada, M., Kawachi, I., Tsujihata, M., Hozumi, I., Morita, T., Onodera, O., et al. (2008) · Acta Neuropathologica · doi:10.1007/s00401-008-0443-6 · Cited by 61
TDP-43 Pathologic Lesions and Clinical Phenotype in Frontotemporal Lobar Degeneration With Ubiquitin-Positive Inclusions
Grossman, M., Wood, E. M., Moore, P., Neumann, M., Kwong, L. K., Forman, M. S., Clark, C. M., McCluskey, L. F., Miller, B. L., Lee, V. M. ‐., et al. (2007) · Archives of Neurology · doi:10.1001/archneur.64.10.1449 · Cited by 60
Self-citation
Amyotrophic Lateral Sclerosis–Plus Syndrome With TAR DNA-Binding Protein-43 Pathology
McCluskey, L. F., Elman, L. B., Martinez‐Lage, M., Deerlin, V. M. V., Yuan, W., Falcone, D., Siderowf, A. D., & Trojanowski, J. Q. (2009) · Archives of Neurology · doi:10.1001/archneur.66.1.121 · Cited by 58
Self-citation
TDP-43 M311V mutation in familial amyotrophic lateral sclerosis
Lemmens, R., Race, V., Hersmus, N., Matthijs, G., Bosch, L. V. D., Damme, P. V., Dubois, B., Boonen, S. E., Goris, A., & Robberecht, W. (2009) · Journal of Neurology Neurosurgery & Psychiatry · doi:10.1136/jnnp.2008.157677 · Cited by 57
Fine structural analysis of the neuronal inclusions of frontotemporal lobar degeneration with TDP-43 proteinopathy
Thorpe, J. R., Tang, H., Atherton, J., & Cairns, N. J. (2008) · Journal of Neural Transmission · doi:10.1007/s00702-008-0137-1 · Cited by 57
Screening for TARDBP mutations in Japanese familial amyotrophic lateral sclerosis
Kamada, M., Maruyama, H., Tanaka, E. I., Morino, H., Wate, R., Ito, H., Kusaka, H., Kawano, Y., Miki, T., Nodera, H., et al. (2009) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2009.04.017 · Cited by 55
Evidence That TDP-43 is Not the Major Ubiquitinated Target Within the Pathological Inclusions of Amyotrophic Lateral Sclerosis
Sanelli, T. R., Xiao, S., Horne, P., Bilbao, J. M., Zinman, L. H., & Robertson, J. (2007) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/nen.0b013e31815c5edd · Cited by 49
TDP‐43 neuropathology is similar in sporadic amyotrophic lateral sclerosis with or without TDP‐43 mutations
Pamphlett, R., Luquin, N., McLean, C., Jew, S. K., & Adams, L. (2008) · Neuropathology and Applied Neurobiology · doi:10.1111/j.1365-2990.2008.00982.x · Cited by 47
Phosphorylated Smad2/3 immunoreactivity in sporadic and familial amyotrophic lateral sclerosis and its mouse model
Nakamura, M., Ito, H., Wate, R., Nakano, S., Hirano, A., & Kusaka, H. (2008) · Acta Neuropathologica · doi:10.1007/s00401-007-0337-z · Cited by 45
TDP-43 gene analysis in frontotemporal lobar degeneration
Rollinson, S. J., Snowden, J. S., Neary, D., Morrison, K. E., Mann, D. M. A., & Pickering-Brown, S. M. (2007) · Neuroscience Letters · doi:10.1016/j.neulet.2007.03.044 · Cited by 44
Ubiquitin associated protein 1 is a risk factor for frontotemporal lobar degeneration
Rollinson, S. J., Rizzu, P., Sikkink, S. K., Baker, M., Halliwell, N. A., Snowden, J. S., Traynor, B. J., Ruano, D., Cairns, N. J., Rohrer, J. D., et al. (2009) · Neurobiology of Aging · doi:10.1016/j.neurobiolaging.2009.01.009 · Cited by 41
Sporadic amyotrophic lateral sclerosis: Widespread multisystem degeneration with TDP‐43 pathology in a patient after long‐term survival on a respirator
Nishihira, Y., Tan, C., Toyoshima, Y., Yonemochi, Y., Kondo, H., Nakajima, T., & Takahashi, H. (2009) · Neuropathology · doi:10.1111/j.1440-1789.2008.00999.x · Cited by 39
No association of TDP-43 with sporadic frontotemporal dementia
Schumacher, A., Friedrich, P., Diehl‐Schmid, J., Ibach, B., Perneczky, R., Eisele, T., Vukovich, R., Förstl, H., & Riemenschneider, M. J. (2007) · Neurobiology of Aging · doi:10.1016/j.neurobiolaging.2007.05.022 · Cited by 39
TDP-43 in skeletal muscle of patients affected with amyotrophic lateral sclerosis
Sorarú, G., Orsetti, V., Buratti, E., Baralle, F. E., Cima, V., Volpe, M., D’Ascenzo, C., Palmieri, A., Koutsikos, K., Pegoraro, E., et al. (2009) · Amyotrophic Lateral Sclerosis · doi:10.3109/17482960902810890 · Cited by 29
Neuropathology of Olfactory Ensheathing Cell Transplantation into the Brain of Two Amyotrophic Lateral Sclerosis (ALS) Patients
Giordana, M. T., Grifoni, S., Votta, B., Magistrello, M., Vercellino, M., Pellerino, A., Navone, R., Valentini, C., Calvo, A., & Chiò, A. (2009) · Brain Pathology · doi:10.1111/j.1750-3639.2009.00353.x · Cited by 26
TDP-43 immunoreactivity in neurodegenerative disorders: disease versus mechanism specificity
Dickson, D. W. (2007) · Acta Neuropathologica · doi:10.1007/s00401-007-0323-5 · Cited by 24
TDP-43 inclusions do not protect motor neurons from sporadic ALS
Pamphlett, R., & Jew, S. K. (2008) · Acta Neuropathologica · doi:10.1007/s00401-008-0392-0 · Cited by 22
TAR DNA-binding protein-43 in amyotrophic lateral sclerosis, frontotemporal lobar degeneration, and Alzheimer disease
Bigio, E. H. (2008) · Acta Neuropathologica · doi:10.1007/s00401-008-0405-z · Cited by 16
Motor Neuron Disease and Frontotemporal Lobar Degeneration: A Tale of Two Disorders Linked To TDP-43
Elman, L. B., McCluskey, L. F., & Grossman, M. (2007) · Neurosignals · doi:10.1159/000109762 · Cited by 14
TDP-43 in skeletal muscle of patients affected with amyotrophic lateral sclerosis
Sorarú, G., Orsetti, V., Buratti, E., Baralle, F. E., Cima, V., Volpe, M., D’Ascenzo, C., Palmieri, A., Koutsikos, K., Pegoraro, E., et al. (2009) · Amyotrophic Lateral Sclerosis · doi:10.1080/17482960902810890 · Cited by 3

Research works printed but not linked: 1

Geser, 2007, The Molecular and genetic Basis of Neurological and Psychiatric Disease, 330

Sorted by citations

Gains or losses: molecular mechanisms of TDP43-mediated neurodegeneration
Lee, E. B., Lee, V. M.-Y., & Trojanowski, J. Q. (2012) · Nature Reviews Neuroscience · doi:10.1038/nrn3121 · Cited by 659
Self-citation
Amyotrophic lateral sclerosis—a model of corticofugal axonal spread
Braak, H., Brettschneider, J., Ludolph, A. C., Lee, V. M., Trojanowski, J. Q., & Tredici, K. D. (2013) · Nature Reviews Neurology · doi:10.1038/nrneurol.2013.221 · Cited by 544
Self-citation
Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice
Igaz, L. M., Kwong, L. K., Lee, E. B., Chen‐Plotkin, A. S., Swanson, E., Unger, T. L., Malunda, J. A., Xu, Y., Winton, M. J., Trojanowski, J. Q., et al. (2011) · Journal of Clinical Investigation · doi:10.1172/jci44867 · Cited by 450
Self-citation
Changes in cognition and behaviour in amyotrophic lateral sclerosis: nature of impairment and implications for assessment
Goldstein, L. H., & Abrahams, S. (2013) · The Lancet Neurology · doi:10.1016/s1474-4422(13)70026-7 · Cited by 439
TDP-43 Is Directed to Stress Granules by Sorbitol, a Novel Physiological Osmotic and Oxidative Stressor
Dewey, C. M., Cenik, B., Sephton, C. F., Dries, D. R., Mayer, P. R., Rich, S. K., Johnson, B. A., Herz, J., & Yu, G. (2010) · Molecular and Cellular Biology · doi:10.1128/mcb.01279-10 · Cited by 370

See all 195

Authors: Geser, F., Lee, V. M.-Y., & Trojanowski, J. Q.
Affiliations: University of Pennsylvania (US)
Corresponding author: Trojanowski, John Q.
ORCID: Geser, Felix
Lee, Virginia M.-Y.
Trojanowski, John Q.
Venue: Neuropathology
Publisher: Wiley
Published: 2010-04
Volume: 30
Issue: 2
Pages: 103-112
ISSN: 0919-6544 (ISSN-L)
1440-1789
DOI: 10.1111/j.1440-1789.2009.01091.x
PMC: PMC3131978
OpenAlex: W2125931517
Cited by: 195
References: 108
Citations by year: 2025: 4 · 2024: 11 · 2023: 15 · 2022: 11 · 2021: 8 · 2020: 8 · 2019: 12 · 2018: 8 · 2017: 10 · 2016: 10 · 2015: 12 · 2014: 10 · 2013: 16 · 2012: 24
FWCI: 3.01
Type: journal article
Language: en
Open access: green
www.ncbi.nlm.nih.gov
Licence: doi.wiley.com/10.1002/tdm_license_1.1 (tdm, 1979 d)
Locations: Neuropathology (publishedVersion)
PubMed (publishedVersion)
PubMed Central (acceptedVersion)
Funders: National Institute on Aging (P30AG010124)
Topics: Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
Health Sciences › Medicine › Internal Medicine › Amyloidosis: Diagnosis, Treatment, Outcomes
Keywords: amyotrophic lateral sclerosis, frontotemporal lobar degeneration, TDP-43, TDP-43 proteinopathy, FTLD-TDP, disease continuum, neurodegenerative diseases, synucleinopathies, tauopathy, ubiquitin-positive inclusions
MeSH terms: Amyotrophic Lateral Sclerosis, Brain, DNA-Binding Proteins, Humans, Neurons, Frontotemporal Lobar Degeneration

About these checks

Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.

Statistics and arithmetic are recomputed, not judged.

Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.

What counts as a claim ›How we measure confidence ›How we check this ›

Are you an author, or do you see a problem with this page? Request a review

Amyotrophic lateral sclerosis and frontotemporal lobar degeneration: A spectrum of TDP-43 proteinopathies

Abstract

Overview

Integrity signals

Verification

Claims the paper makes

Claims other papers make about this one

Self-check

Citations

Cites

Cited by

Related

Metadata

Record

About these checks