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Article

Protein disulphide isomerase (PDI) is protective against amyotrophic lateral sclerosis (ALS)-related mutant Fused in Sarcoma (FUS) in in vitro models.

Scientific reports - 2 Sept 2021

Parakh S, Perri E R, Vidal M, Sultana J, Shadfar S, Mehta P, Konopka A, Thomas C J, Spencer D M, Atkin J D

Abstract excerpt

Mutations in Fused in Sarcoma (FUS) are present in familial and sporadic cases of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD). FUS is localised in the nucleus where it has important functions in DNA repair. However, in ALS/FTD, mutant FUS mislocalises from the nucleus to the cytoplasm where it forms inclusions, a key pathological hallmark of neurodegeneration. Mutant FUS also inhibits...

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Identifiers

PMID: 34475430
DOI: 10.1038/s41598-021-96181-2
PMCID: PMC8413276

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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