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Article

Allele-specific gene silencing in two mouse models of autosomal dominant skeletal myopathy.

PloS one - 1 Jan 2012

Loy Ryan E, Lueck John D, Mostajo-Radji Mohammed A, Carrell Ellie M, Dirksen Robert T

Abstract excerpt

We explored the potential of mutant allele-specific gene silencing (ASGS) in providing therapeutic benefit in two established mouse models of the autosomal dominantly-inherited muscle disorders, Malignant Hyperthermia (MH) and Central Core Disease (CCD). Candidate ASGS siRNAs were designed and validated for efficacy and specificity on ryanodine receptor (RyR1) cDNA mini-constructs expressed in HEK293 cells using...

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Identifiers

PMID: 23152933
DOI: 10.1371/journal.pone.0049757
PMCID: PMC3495761

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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