Article
Proteasome inhibition improves the muscle of laminin α2 chain-deficient mice.
Human molecular genetics - 1 Feb 2011
Carmignac Virginie, Quéré Ronan, Durbeej Madeleine
Abstract excerpt
Muscle atrophy, a significant characteristic of congenital muscular dystrophy with laminin α2 chain deficiency (also known as MDC1A), occurs by a change in the normal balance between protein synthesis and protein degradation. The ubiquitin-proteasome system (UPS) plays a key role in protein degradation in skeletal muscle cells. In order to identify new targets for drug therapy against MDC1A, we have investigated...
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