Pathological TDP‐43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutations
Open accessAbstract
Objective: Amyotrophic lateral sclerosis (ALS) is a common, fatal motor neuron disorder with no effective treatment. Approximately 10% of cases are familial ALS (FALS), and the most common genetic abnormality is superoxide dismutase ‐ 1 ( SOD1 ) mutations. Most ALS research in the past decade has focused on the neurotoxicity of mutant SOD1, and this knowledge has directed therapeutic strategies. We recently identified TDP‐43 as the major pathological protein in sporadic ALS. In this study, we investigated TDP‐43 in a larger series of ALS cases (n = 111), including familial cases with and without SOD1 mutations. Methods: Ubiquitin and TDP‐43 immunohistochemistry was performed on postmortem tissue from sporadic ALS (n = 59), ALS with SOD1 mutations (n = 15), SOD ‐ 1 –negative FALS (n = 11), and ALS with dementia (n = 26). Biochemical analysis was performed on representative cases from each group. Results: All cases of sporadic ALS, ALS with dementia, and SOD1 ‐negative FALS had neuronal and glial inclusions that were immunoreactive for both ubiquitin and TDP‐43. Cases with SOD1 mutations had ubiquitin‐positive neuronal inclusions; however, no cases were immunoreactive for TDP‐43. Biochemical analysis of postmortem tissue from sporadic ALS and SOD1 ‐negative FALS demonstrated pathological forms of TDP‐43 that were absent in cases with SOD1 mutations. Interpretation: These findings implicate pathological TDP‐43 in the pathogenesis of sporadic ALS. In contrast, the absence of pathological TDP‐43 in cases with SOD1 mutations implies that motor neuron degeneration in these cases may result from a different mechanism, and that cases with SOD1 mutations may not be the familial counterpart of sporadic ALS. Ann Neurol 2007;61:427–434
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Oligodendrocytes in ALS patients demonstrate inclusions containing TARDBP and FUS.
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- Authors
- Mackenzie, I. R. A., Bigio, E. H., Ince, P. G., Geser, F., Neumann, M., Cairns, N. J., Kwong, L. K., Forman, M. S., Ravits, J., Stewart, H., Eisen, A., McClusky, L., Kretzschmar, H. A., Monoranu, C. M., Highley, J. R., Kirby, J., Siddique, T., Shaw, P. J., Lee, V. M., & Trojanowski, J. Q.
- Affiliations
- University of British Columbia (CA)
- Northwestern University (US)
- University of Sheffield (GB)
- University of Pennsylvania (US)
- Institut für Neuropathologie (DE)
- Washington University in St. Louis (US)
- Virginia Mason Medical Center (US)
- University of Würzburg (DE)
- Corresponding author
- Mackenzie, Ian R. A.
- ORCID
- Venue
- Annals of Neurology
- Publisher
- Wiley
- Published
- 2007-04-27
- Volume
- 61
- Issue
- 5
- Pages
- 427-434
- ISSN
- 0364-5134 (ISSN-L)
- 1531-8249
- OpenAlex
- W2053362200
- Cited by
- 992
- References
- 34
- Citations by year
- 2026: 23 · 2025: 55 · 2024: 57 · 2023: 45 · 2022: 59 · 2021: 61 · 2020: 53 · 2019: 46 · 2018: 57 · 2017: 50 · 2016: 50 · 2015: 43 · 2014: 36 · 2013: 49 · 2012: 54
- FWCI
- 44.73
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Annals of Neurology (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Wellcome Trust
- National Institutes of Health
- Canadian Institutes of Health Research
- National Institute on Aging (AG13854, P30AG10124, U01AG016976, P01 AG-17586, P30AG010124, P01AG017586, U01-AG 16976, P 50 AG05681, P50AG005681, P30AG013854)
- Topics
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Health Sciences › Medicine › Physiology › Alzheimer's disease research and treatments
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Hereditary Neurological Disorders
- Keywords
- amyotrophic lateral sclerosis, TDP-43, SOD1 mutations, sporadic ALS, familial ALS, ubiquitin-positive inclusions, motor neuron degeneration, ALS pathogenesis
- MeSH terms
- Superoxide Dismutase-1, Amyotrophic Lateral Sclerosis, DNA-Binding Proteins, Humans, Immunohistochemistry, Medulla Oblongata, Motor Cortex, Mutation, Spinal Cord, Superoxide Dismutase, Ubiquitin
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 ›
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