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The potential of sarcospan in adhesion complex replacement therapeutics for the treatment of muscular dystrophy
Author(s) -
Marshall Jamie L.,
Kwok Yukwah,
McMorran Brian J.,
Baum Linda G.,
CrosbieWatson Rachelle H.
Publication year - 2013
Publication title -
the febs journal
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.981
H-Index - 204
eISSN - 1742-4658
pISSN - 1742-464X
DOI - 10.1111/febs.12295
Subject(s) - muscular dystrophy , utrophin , dystrophin , sarcolemma , itga7 , extracellular matrix , duchenne muscular dystrophy , microbiology and biotechnology , regeneration (biology) , adhesion , actin , biology , myocyte , medicine , chemistry , genetics , organic chemistry
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.