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tdp-43
TDP-43
TDP-43 (TARDBP) is a nuclear RNA-binding protein that regulates pre-mRNA splicing and stability; nuclear clearance and cytoplasmic ubiquitinated inclusions are the pathological hallmark of >97% of ALS and ~45% of FTLD; TARDBP mutations cause ~4% of familial ALS.
Entry Metadata
| Field | Value |
|---|---|
| ID | tdp-43 |
| Name | TDP-43 |
| Status | draft |
| Last reviewed | 2026-06-08 |
| Atlas | 01-human |
| Scale | 03-molecular |
Cross-Atlas Connections
Sources
- Neumann M, Sampathu DM, Kwong LK, et al. Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis. Science. 2006;314(5796):130-133. · PubMed 17023659
- Sreedharan J, Blair IP, Tripathi VB, et al. TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis. Science. 2008;319(5870):1668-1672. · PubMed 18309045
- Prasad A, Bharathi V, Sivalingam V, Girdhar A, Patel BK. Molecular mechanisms of TDP-43 misfolding and pathology in amyotrophic lateral sclerosis. Front Mol Neurosci. 2019;12:25. · PubMed 30837838