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Article

Effects of Cellular Pathway Disturbances on Misfolded Superoxide Dismutase-1 in Fibroblasts Derived from ALS Patients.

PloS one - 1 Jan 2016

Keskin Isil, Forsgren Elin, Lange Dale J, Weber Markus, Birve Anna, Synofzik Matthis, Gilthorpe Jonathan D, Andersen Peter M, Marklund Stefan L

Abstract excerpt

Mutations in superoxide dismutase-1 (SOD1) are a common known cause of amyotrophic lateral sclerosis (ALS). The neurotoxicity of mutant SOD1s is most likely caused by misfolded molecular species, but disease pathogenesis is still not understood. Proposed mechanisms include impaired mitochondrial function, induction of endoplasmic reticulum stress, reduction in the activities of the proteasome and autophagy, and...

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Identifiers

PMID: 26919046
DOI: 10.1371/journal.pone.0150133
PMCID: PMC4769150

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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