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Clinical and Pathological Continuum of Multisystem TDP-43 Proteinopathies
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OBJECTIVE: To determine the extent of transactivation response DNA-binding protein with a molecular weight of 43 kDa (TDP-43) pathology in the central nervous system of patients with clinically and autopsy-confirmed diagnoses of frontotemporal lobar degeneration with and without motor neuron disease and amyotrophic lateral sclerosis with and without cognitive impairment. DESIGN: Performance of immunohistochemical whole-central nervous system scans for evidence of pathological TDP-43 and retrospective clinical medical record review. SETTING: An academic medical center. PARTICIPANTS: We included 64 patients with clinically and pathologically confirmed frontotemporal lobar degeneration with ubiquitinated inclusions with or without motor neuron disease and amyotrophic lateral sclerosis with or without cognitive impairment. MAIN OUTCOME MEASURE: Neuronal and glial TDP-43 pathology. RESULTS: We found evidence of neuronal and glial TDP-43 pathology in all disease groups throughout the neuraxis, albeit with variations in the frequency, morphology, and distribution of TDP-43 lesions. Moreover, the major clinical manifestations (eg, cognitive impairments, motor neuron signs, extrapyramidal symptoms, neuropsychiatric features) were reflected by the predominant distribution and burden of TDP-43 pathology. CONCLUSION: These findings strongly suggest that amyotrophic lateral sclerosis, frontotemporal lobar degeneration with amyotrophic lateral sclerosis or motor neuron disease, and frontotemporal lobar degeneration with ubiquitinated inclusions are different manifestations of a multiple-system TDP-43 proteinopathy linked to similar mechanisms of neurodegeneration.
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“TDP‐43 inclusions are also observed in > 95% of ALS, 22 supporting the notion that FTLD‐TDP and ALS are part of a clinicopathological continuum.”
From New developments of biofluid‐based biomarkers for routine diagnosis and disease trajectories in frontotemporal dementia (2022) · cites this paper as [23]
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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-citationEl 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
Self-citationWeıntraub, S. (1990) · Archives of Neurology · doi:10.1001/archneur.1990.00530120075013 · Cited by 3,018
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-citationFrontotemporal lobar degeneration: A consensus on clinical diagnostic criteria
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Morita, M., Al‐Chalabi, A., Andersen, P. M., Hosler, B. A., Sapp, P. C., Englund, E. M., Mitchell, J. E., Habgood, J., de Belleroche, J., Xi, J., et al. (2006) · Neurology · doi:10.1212/01.wnl.0000200048.53766.b4 · Cited by 363
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
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
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Khosravi, B., LaClair, K. D., Riemenschneider, H., Zhou, Q., Frottin, F., Mareljic, N., Czuppa, M., Farny, D., Hartmann, H., Michaelsen, M., et al. (2020) · The EMBO Journal · doi:10.15252/embj.2019102811 · Cited by 70
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Trojsi, F., Esposito, F., Stefano, M. D., Buonanno, D., Conforti, F. L., Corbo, D., Piccirillo, G., Cirillo, M., Monsurrò, M. R., Montella, P., et al. (2014) · Neurobiology of Aging · doi:10.1016/j.neurobiolaging.2014.06.025 · Cited by 72
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Halabi, C., Halabi, A., Dean, D. L., Wang, P., Boxer, A. L., Trojanowski, J. Q., DeArmond, S. J., Miller, B. L., Kramer, J. H., & Seeley, W. W. (2012) · Alzheimer Disease & Associated Disorders · doi:10.1097/wad.0b013e31824a7df4 · Cited by 73
Self-citationRepetitive elements in aging and neurodegeneration
Copley, K. E., & Shorter, J. (2023) · Trends in Genetics · doi:10.1016/j.tig.2023.02.008 · Cited by 70
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Tada, M., Coon, E. A., Osmand, A. P., Kirby, P., Martin, W. R. W., Wieler, M., Shiga, A., Shirasaki, H., Tada, M., Makifuchi, T., et al. (2012) · Acta Neuropathologica · doi:10.1007/s00401-012-1005-5 · Cited by 69
Braak, H., Ludolph, A. C., Thal, D. R., & Tredici, K. D. (2010) · Acta Neuropathologica · doi:10.1007/s00401-010-0683-0 · Cited by 67
Gordon, P. H., Delgadillo, D., Piquard, A., Bruneteau, G., Pradat, P., Salachas, F., Payan, C., Meininger, V., & Lacomblez, L. (2011) · Amyotrophic Lateral Sclerosis · doi:10.3109/17482968.2011.580847 · Cited by 67
Cirillo, M., Esposito, F., Tedeschi, G., Caiazzo, G., Sagnelli, A., Piccirillo, G., Conforti, R., Tortora, F., Monsurrò, M. R., Cirillo, S., et al. (2012) · American Journal of Neuroradiology · doi:10.3174/ajnr.a2918 · Cited by 66
Porta, S., Xu, Y., Lehr, T., Zhang, B., Meymand, E. S., Olufemi, M. F., Stieber, A., Lee, E. B., Trojanowski, J. Q., & Lee, V. M. ‐. (2021) · Neuropathology and Applied Neurobiology · doi:10.1111/nan.12732 · Cited by 58
Self-citationBarmada, S. J., & Finkbeiner, S. M. (2010) · Reviews in the Neurosciences · doi:10.1515/revneuro.2010.21.4.251 · Cited by 63
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Takeuchi, R., Tada, M., Shiga, A., Toyoshima, Y., Konno, T., Sato, T., Nozaki, H., Kato, T., Horie, M., Shimizu, H., et al. (2016) · Acta Neuropathologica Communications · doi:10.1186/s40478-016-0335-2 · Cited by 66
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Maniecka, Z., & Polymenidou, M. (2015) · Virus Research · doi:10.1016/j.virusres.2014.12.032 · Cited by 65
Gelon, P. A., Dutchak, P. A., & Sephton, C. F. (2022) · Frontiers in Molecular Neuroscience · doi:10.3389/fnmol.2022.1000183 · Cited by 64
Osborne, R. A., Sekhon, R., Johnston, W. S. W., & Kalra, S. (2013) · Journal of the Neurological Sciences · doi:10.1016/j.jns.2013.10.038 · Cited by 59
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Štětkářová, I., & Ehler, E. (2021) · Diagnostics · doi:10.3390/diagnostics11020231 · Cited by 59
Geser, F., Prvulovic, D., O’Dwyer, L., Hardiman, O. M., Bede, P., Bokde, A. L. W., Trojanowski, J. Q., & Hampel, H. J. (2011) · Progress in Neurobiology · doi:10.1016/j.pneurobio.2011.08.011 · Cited by 58
Self-citationMcGurk, L., Lee, V. M., Trojanowksi, J. Q., Deerlin, V. M. V., Lee, E. B., & Bonini, N. M. (2014) · Journal of Neuropathology & Experimental Neurology · doi:10.1097/nen.0000000000000102 · Cited by 57
Self-citationBorghero, G., Floris, G., Cannas, A., Marrosu, M. G., Murru, M. R., Costantino, E., Parish, L. D., Pugliatti, M., Ticca, A., Traynor, B. J., et al. (2011) · Neurobiology of Aging · doi:10.1016/j.neurobiolaging.2011.06.009 · Cited by 57
White matter imaging contributes to the multimodal diagnosis of frontotemporal lobar degeneration
McMillan, C. T., Brun, C., Siddiqui, S. M., Churgin, M., Libon, D. J., Yushkevich, P. A., Zhang, H. G., Boller, A., Gee, J. C., & Grossman, M. (2012) · Neurology · doi:10.1212/wnl.0b013e31825830bd · Cited by 55
Self-citationdel Campo, M., Zetterberg, H., Gandy, S., Onyike, C. U., Oliveira, F., Udeh‐Momoh, C., Lleó, A., Teunissen, C. E., & Pijnenburg, Y. (2022) · Alzheimer's & Dementia · doi:10.1002/alz.12643 · Cited by 52
Bourinaris, T., & Houlden, H. (2018) · Movement Disorders Clinical Practice · doi:10.1002/mdc3.12677 · Cited by 58
Interplay between the ubiquitin-proteasome system and autophagy in proteinopathies.
Zheng, Q., Li, J., & Wang, X. (2009) · Europe PMC (PubMed Central) · Cited by 54
Kamada, M., Izumi, Y., Ayaki, T., Nakamura, M., Kagawa, S., Kudo, E., Sako, W., Maruyama, H., Nishida, Y., Kawakami, H., et al. (2013) · Neuropathology · doi:10.1111/neup.12051 · Cited by 56
Keeney, P. M., & Bennett, J. P. (2010) · Molecular Neurodegeneration · doi:10.1186/1750-1326-5-21 · Cited by 51
Metadata
- Authors
- Geser, F., Martinez-Lage, M., Robinson, J., Uryu, K., Neumann, M., Brandmeir, N. J., Xie, S. X., Kwong, L. K., Elman, L., McCluskey, L., Clark, C. M., Malunda, J., Miller, B. L., Zimmerman, E. A., Qian, J., Van Deerlin, V., Grossman, M., Lee, V. M.-Y., & Trojanowski, J. Q.
- Affiliations
- University of Pennsylvania (US)
- Universitat Autònoma de Barcelona (ES)
- Corresponding author
- Trojanowski, John Q.
- ORCID
- Venue
- Archives of Neurology
- Publisher
- American Medical Association (AMA)
- Published
- 2009-02-01
- Volume
- 66
- Issue
- 2
- Pages
- 180-9
- ISSN
- 0003-9942 (ISSN-L)
- PMC
- PMC2774117
- OpenAlex
- W2117597459
- Cited by
- 267
- References
- 19
- Citations by year
- 2026: 7 · 2025: 13 · 2024: 3 · 2023: 16 · 2022: 11 · 2021: 12 · 2020: 12 · 2019: 9 · 2018: 12 · 2017: 11 · 2016: 11 · 2015: 9 · 2014: 19 · 2013: 21 · 2012: 29
- FWCI
- 21.55
- Type
- journal article
- Language
- en
- Open access
- green
- www.ncbi.nlm.nih.gov
- Locations
- Archives of Neurology (publishedVersion)
- PubMed (publishedVersion)
- PubMed Central (acceptedVersion)
- Funders
- National Institutes of Health (AG 10 124, AG 17586)
- National Institute on Aging (P30AG010124, P01AG017586, AG17586, AG-10124, P01AG019724)
- National Heart, Lung, and Blood Institute (T35HL071483, HL071483)
- 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 › Psychiatry and Mental health › Dementia and Cognitive Impairment Research
- Keywords
- TDP-43 proteinopathy, frontotemporal lobar degeneration, amyotrophic lateral sclerosis, motor neuron disease, ubiquitinated inclusions, cognitive impairment, extrapyramidal symptoms, neuropsychiatric features, neurodegeneration, clinicopathological correlation
- MeSH terms
- Aged, Amyotrophic Lateral Sclerosis, Brain, Brain Mapping, Inclusion Bodies, Cognition Disorders, Dementia, DNA-Binding Proteins, Female, Humans, Immunohistochemistry, Male, Middle Aged, Movement Disorders, Neuroglia, Neurons, Retrospective Studies, Motor Neuron Disease, Disease Progression, Ubiquitin, Ubiquitination
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