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Article

Endoplasmic reticulum stress leads to accumulation of wild-type SOD1 aggregates associated with sporadic amyotrophic lateral sclerosis.

Proceedings of the National Academy of Sciences of the United States of America - 7 Aug 2018

Medinas Danilo B, Rozas Pablo, Martínez Traub Francisca, Woehlbier Ute, Brown Robert H, Bosco Daryl A, Hetz Claudio

Abstract excerpt

Abnormal modifications to mutant superoxide dismutase 1 (SOD1) are linked to familial amyotrophic lateral sclerosis (fALS). Misfolding of wild-type SOD1 (SOD1WT) is also observed in postmortem tissue of a subset of sporadic ALS (sALS) cases, but cellular and molecular mechanisms generating abnormal SOD1WT species are unknown. We analyzed aberrant human SOD1WT species over the lifetime of transgenic mice and found...

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Identifiers

PMID: 30038021
DOI: 10.1073/pnas.1801109115
PMCID: PMC6094144

Genes

SOD1

Provenance

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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