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

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 adipogenes...

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Bibliographische Detailangaben
Veröffentlicht in:EBioMedicine
Hauptverfasser: Noguchi, Satoru, Ogawa, Megumu, Malicdan, May Christine, Nonaka, Ikuya, Nishino, Ichizo
Format: Artigo
Sprache:Inglês
Veröffentlicht: Elsevier 2016
Schlagworte:
Research Paper
Online Zugang:https://ncbi.nlm.nih.gov/pmc/articles/PMC5233815/
https://ncbi.nlm.nih.gov/pubmed/28043812
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.ebiom.2016.12.011
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