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ISSN 2992-779X (Electronic)
ISSN 2007-6452 (Print)

2016, Number 1

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Investigación en Discapacidad 2016; 5 (1)

The sarcospan-sarcoglycan subcomplex: its importance in striated muscle and vascular tissue

Coral-Vázquez RM, Ramírez BR, Palma-Flores C, Ramírez-Sánchez I, De los Santos S, Canto P

Language: Spanish
References: 43
Page: 39-45
PDF size: 340.70 Kb.


ABSTRACT

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 during contraction the process. Additionally, there are evidences of their participation in signal transduction mechanisms. Mutations in these proteins are the cause of limb girdle muscular dystrophy 2C-F (LGMD 2C-F). Other isoforms of SGs, ε- and ζ-, have not been associated 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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