Proteasome Inhibitor (MG-132) Treatment of mdx Mice Rescues the Expression and Membrane Localization of Dystrophin and Dystrophin-Associated Proteins

Dystrophin, the protein product of the Duchenne muscular dystrophy (DMD) gene, is absent in the skeletal muscle of DMD patients and mdx mice. At the plasma membrane of skeletal muscle fibers, dystrophin associates with a multimeric protein complex, termed the dystrophin-glycoprotein complex (DGC). P...

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Main Authors: Bonuccelli, Gloria, Sotgia, Federica, Schubert, William, Park, David S., Frank, Philippe G., Woodman, Scott E., Insabato, Luigi, Cammer, Michael, Minetti, Carlo, Lisanti, Michael P.
Format: Artigo
Jezik:Inglês
Izdano: American Society for Investigative Pathology 2003
Teme:
Animal Model
Online dostop:https://ncbi.nlm.nih.gov/pmc/articles/PMC1868305/
https://ncbi.nlm.nih.gov/pubmed/14507673
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