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The potential of sarcospan in adhesion complex replacement therapeutics for the treatment of muscular dystrophy.
- Source :
- The FEBS journal; vol 280, iss 17, 4210-4229; 1742-464X
- Publication Year :
- 2013
-
Abstract
- Three adhesion complexes span the sarcolemma and facilitate critical connections between the extracellular matrix and the actin cytoskeleton: the dystrophin- and utrophin-glycoprotein complexes and α7β1 integrin. Loss of individual protein components results in a loss of the entire protein complex and muscular dystrophy. Muscular dystrophy is a progressive, lethal wasting disease characterized by repetitive cycles of myofiber degeneration and regeneration. Protein-replacement therapy offers a promising approach for the treatment of muscular dystrophy. Recently, we demonstrated that sarcospan facilitates protein-protein interactions amongst the adhesion complexes and is an important potential therapeutic target. Here, we review current protein-replacement strategies, discuss the potential benefits of sarcospan expression, and identify important experiments that must be addressed for sarcospan to move to the clinic.
Details
- Database :
- OAIster
- Journal :
- The FEBS journal; vol 280, iss 17, 4210-4229; 1742-464X
- Notes :
- Marshall, Jamie L, Kwok, Yukwah, McMorran, Brian J, Baum, Linda G, Crosbie-Watson, Rachelle H
- Publication Type :
- Electronic Resource
- Accession number :
- edsoai.on1277079927
- Document Type :
- Electronic Resource