Article
Autophagy is increased in laminin α2 chain-deficient muscle and its inhibition improves muscle morphology in a mouse model of MDC1A.
Human molecular genetics - 15 Dec 2011
Carmignac Virginie, Svensson Martina, Körner Zandra, Elowsson Linda, Matsumura Cintia, Gawlik Kinga I, Allamand Valerie, Durbeej Madeleine
Abstract excerpt
Congenital muscular dystrophy caused by laminin α2 chain deficiency (also known as MDC1A) is a severe and incapacitating disease, characterized by massive muscle wasting. The ubiquitin-proteasome system plays a major role in muscle wasting and we recently demonstrated that increased proteasomal activity is a feature of MDC1A. The autophagy-lysosome pathway is the other major system involved in degradation of...
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