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Article

Potentiation of amyotrophic lateral sclerosis (ALS)-associated TDP-43 aggregation by the proteasome-targeting factor, ubiquilin 1.

The Journal of biological chemistry - 20 Mar 2009

Kim Sang Hwa, Shi Yuling, Hanson Keith A, Williams Leah M, Sakasai Ryo, Bowler Michael J, Tibbetts Randal S

Abstract excerpt

TDP-43 (43-kDa TAR DNA-binding domain protein) is a major constituent of ubiquitin-positive cytoplasmic aggregates present in neurons of patients with fronto-temporal lobular dementia and amyotrophic lateral sclerosis (ALS). The pathologic significance of TDP-43 aggregation is not known; however, dominant mutations in TDP-43 cause a subset of ALS cases, suggesting that misfolding and/or altered trafficking of...

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Source

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Identifiers

PMID: 19112176
DOI: 10.1074/jbc.M808064200
PMCID: PMC2658102

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 5 Sept 2026. Manifest publication-graph-cohort-2026-09-05.1.

Source receipt: pubmed, retrieved 5 Sept 2026.

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