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Article

Oxidative stress and successful antioxidant treatment in models of RYR1-related myopathy.

Brain : a journal of neurology - 1 Apr 2012

Dowling James J, Arbogast Sandrine, Hur Junguk, Nelson Darcee D, McEvoy Anna, Waugh Trent, Marty Isabelle, Lunardi Joel, Brooks Susan V, Kuwada John Y, Ferreiro Ana

Abstract excerpt

The skeletal muscle ryanodine receptor is an essential component of the excitation-contraction coupling apparatus. Mutations in RYR1 are associated with several congenital myopathies (termed RYR1-related myopathies) that are the most common non-dystrophic muscle diseases of childhood. Currently, no treatments exist for these disorders. Although the primary pathogenic abnormality involves defective...

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Source

Open in PubMed Open full text

Identifiers

PMID: 22418739
DOI: 10.1093/brain/aws036
PMCID: PMC3326256

Genes

RYR1

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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