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Article

Spinal cord endoplasmic reticulum stress associated with a microsomal accumulation of mutant superoxide dismutase-1 in an ALS model.

Proceedings of the National Academy of Sciences of the United States of America - 11 Apr 2006

Kikuchi Hitoshi, Almer Gabriele, Yamashita Satoshi, Guégan Christelle, Nagai Makiko, Xu Zuoshang, Sosunov Alexander A, McKhann Guy M, Przedborski Serge

Abstract excerpt

Mutation in superoxide dismutase-1 (SOD1), which is a cause of ALS, alters the folding patterns of this protein. Accumulation of misfolded mutant SOD1 might activate endoplasmic reticulum (ER) stress pathways. Here we show that transgenic mice expressing ALS-linked SOD1 mutants exhibit molecular alterations indicative of a recruitment of ER's signaling machinery. We demonstrate by biochemical and morphological...

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Identifiers

PMID: 16595634
DOI: 10.1073/pnas.0509227103
PMCID: PMC1458691

Genes

SOD1

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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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