The sarcospan-sarcoglycan subcomplex: its importance in striated muscle and vascular tissue
Keywords:
Sarcoglycan complex, striated muscle, vascular tissueAbstract
The sarcoglycans are members of a group of transmembrane proteins closely related to a
larger complex of proteins associated with dystrophin (DAPC, Dystrophin Associated Protein
Complex). Initially, four sarcoglycan were described in the sarcolemma of skeletal muscle
fibers (α-, β-, γ- and δ-SG). The SG subcomplex, along with the protein sarcospan and the
subcomplex dystroglycan (DG), is part of a scaffold that binds the extracellular matrix with
the cytoskeleton. All these proteins together protect the cell from mechanical damage dur-
ing contraction the process. Additionally, there are evidences of their participation in signal
dystrophy 2C-F (LGMD 2C-F). Other isoforms of SGs, ε- and ζ-, have not been associated transduction mechanisms. Mutations in these proteins are the cause of limb girdle muscular
with muscular dystrophy; although mutations in ε-SG can cause diseases such as myoclonic dystonia. Deficiency or absence of β-, γ- and δ-SG has been as well associated with dilated cardiomyopathy. In this regard, various studies have revealed the presence of alternate forms of SG complex in vascular smooth muscle and endothelium, and the relevance of these proteins in vascular physiology.
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