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Article

Intracerebroventricular administration of Cystatin C ameliorates disease in SOD1-linked amyotrophic lateral sclerosis mice.

Journal of neurochemistry - 1 Apr 2018

Watanabe Seiji, Komine Okiru, Endo Fumito, Wakasugi Keisuke, Yamanaka Koji

Abstract excerpt

Cystatin C (CysC) is a major protein component of Bunina bodies, which are a pathological hallmark observed in the remaining motor neurons of patients with amyotrophic lateral sclerosis (ALS). Dominant mutations in the SOD1 gene, encoding Cu/Zn superoxide dismutase (SOD1), are causative for a subset of inherited ALS cases. Our previous study showed that CysC exerts a neuroprotective effect against mutant...

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Identifiers

PMID: 29282717
DOI: 10.1111/jnc.14285
PMCID: PMC5947136

Genes

SOD1
PGC

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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