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Ubiquitinated TDP-43 in Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis
Abstract
Ubiquitin-positive, tau- and α-synuclein–negative inclusions are hallmarks of frontotemporal lobar degeneration with ubiquitin-positive inclusions and amyotrophic lateral sclerosis. Although the identity of the ubiquitinated protein specific to either disorder was unknown, we showed that TDP-43 is the major disease protein in both disorders. Pathologic TDP-43 was hyper-phosphorylated, ubiquitinated, and cleaved to generate C-terminal fragments and was recovered only from affected central nervous system regions, including hippocampus, neocortex, and spinal cord. TDP-43 represents the common pathologic substrate linking these neurodegenerative disorders.
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“In 2006, hyperphosphorylated and ubiquitinated TDP-43 (encoded by TARDBP) cytoplasmic inclusions were identified as the most common (at about 97%) pathological hallmark of ALS.79”
From Role of neuroinflammation in neurodegeneration development (2023) · cites this paper as [79]
In the citing paper
“Degenerating motor neurons in ALS patients commonly display cytoplasmic aggregates containing the DNA/RNA-binding protein TDP-43 (Neumann et al. 2006).”
From Emerging complexity of the HuD/ELAVl4 gene; implications for neuronal development, function, and dysfunction (2013) · cites this paper as Neumann et al. 2006
In the citing paper
“Cytoplasmic TDP43 mislocalization and aggregation are pathological hallmarks of amyotrophic lateral sclerosis (ALS).”
From Nemo-like kinase disrupts nuclear import and drives TDP43 mislocalization in ALS (2025) · cites this paper as [2]
In the citing paper
“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 [22]
In the citing paper
“The scarcity of these samples has prompted efforts to enrich cohorts with FTD syndromes that are highly correlated with a specific underlying neuropathology (e.g., FTD‐ALS for FTLD‐TDP 22 or PSP and CBS for FTLD‐tau 119 , 120 ) and/or to jointly analyze data from both combined cohorts of sporadic and familial cases with the same neuropathology background.”
From New developments of biofluid‐based biomarkers for routine diagnosis and disease trajectories in frontotemporal dementia (2022) · cites this paper as [22]
In the citing paper
“biochemical and histopathologic examination of frontotemporal cortex and spinal cord in sALS patients reveals that ubiquitin-positive inclusions contain TARDBP [274,275]”
From Autophagy and ALS: mechanistic insights and therapeutic implications (2022) · cites this paper as [275]
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Self-citation
Metadata
- Authors
- 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., McCluskey, L. F., Miller, B. L., Masliah, E., Mackenzie, I. R., Feldman, H., Feiden, W., Kretzschmar, H. A., Trojanowski, J. Q., & Lee, V. M.-Y.
- Affiliations
- University of Pennsylvania (US)
- Corresponding author
- Neumann, Manuela
- Sampathu, Deepak M.
- Kwong, Linda K.
- Lee, Virginia M.-Y.
- ORCID
- Venue
- Science
- Published
- 2006-10-06
- Volume
- 314
- Issue
- 5796
- Pages
- 130-133
- ISSN
- 0036-8075 (ISSN-L)
- 1095-9203
- OpenAlex
- W2003618511
- Cited by
- 6,576
- References
- 25
- Citations by year
- 2026: 341 · 2025: 401 · 2024: 411 · 2023: 405 · 2022: 425 · 2021: 441 · 2020: 442 · 2019: 368 · 2018: 400 · 2017: 339 · 2016: 315 · 2015: 283 · 2014: 305 · 2013: 311 · 2012: 301
- FWCI
- 115.98
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- Science (publishedVersion)
- PubMed (publishedVersion)
- Funders
- National Institute on Aging (T32 AG 00255, P30AG010124, P01AG017586, AG17586, AG-10124, P01AG019724, T32AG000255)
- Topics
- Health Sciences › Medicine › Clinical Neurology › Amyotrophic Lateral Sclerosis Research
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Alzheimer's disease research and treatments
- Life Sciences › Neuroscience › Cellular and Molecular Neuroscience › Genetic Neurodegenerative Diseases
- Keywords
- TDP-43, frontotemporal lobar degeneration, amyotrophic lateral sclerosis, ubiquitination, C-terminal fragment, ubiquitin-positive inclusions, neurodegeneration, hippocampus, neocortex, spinal cord
- MeSH terms
- Amino Acid Sequence, Amyotrophic Lateral Sclerosis, Antibodies, Monoclonal, Brain Chemistry, Cerebral Cortex, Dementia, DNA-Binding Proteins, Fluorescent Antibody Technique, Hippocampus, Humans, Molecular Sequence Data, Motor Neurons, Neurons, Peptide Fragments, Phosphorylation, Spinal Cord, Immunoblotting, Ubiquitin
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