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Article

Mutant TDP-43 within motor neurons drives disease onset but not progression in amyotrophic lateral sclerosis.

Acta neuropathologica - 1 Jun 2017

Ditsworth Dara, Maldonado Marcus, McAlonis-Downes Melissa, Sun Shuying, Seelman Amanda, Drenner Kevin, Arnold Eveline, Ling Shuo-Chien, Pizzo Donald, Ravits John, Cleveland Don W, Da Cruz Sandrine

Abstract excerpt

Mutations in TDP-43 cause amyotrophic lateral sclerosis (ALS), a fatal paralytic disease characterized by degeneration and premature death of motor neurons. The contribution of mutant TDP-43-mediated damage within motor neurons was evaluated using mice expressing a conditional allele of an ALS-causing TDP-43 mutant (Q331K) whose broad expression throughout the central nervous system mimics endogenous TDP-43....

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Methodology Data sources Editorial policy Privacy About Contact Helena Bioinformatics

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Source

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Identifiers

PMID: 28357566
DOI: 10.1007/s00401-017-1698-6
PMCID: PMC5427168

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 6 Sept 2026. Manifest publication-graph-cohort-2026-09-06.1.

Source receipt: pubmed, retrieved 6 Sept 2026.

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