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Article

Muscle Weakness and Fibrosis Due to Cell Autonomous and Non-cell Autonomous Events in Collagen VI Deficient Congenital Muscular Dystrophy.

EBioMedicine - 1 Feb 2017

Noguchi Satoru, Ogawa Megumu, Malicdan May Christine, Nonaka Ikuya, Nishino Ichizo

Abstract excerpt

Congenital muscular dystrophies with collagen VI deficiency are inherited muscle disorders with a broad spectrum of clinical presentation and are caused by mutations in one of COL6A1-3 genes. Muscle pathology is characterized by fiber size variation and increased interstitial fibrosis and adipogenesis. In this study, we define critical events that contribute to muscle weakness and fibrosis in a mouse model with...

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Identifiers

PMID: 28043812
DOI: 10.1016/j.ebiom.2016.12.011
PMCID: PMC5233815

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