El subcomplejo sarcoglicano-sarcospan: su importancia en el músculo estriado y tejido vascular
Year: 2016
Type: article
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-, γand 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, eand ζ-, have not been associated with muscular dystrophy; although mutations in e-SG can cause diseases such as myoclonic dystonia. Deficiency or absence of β-, γand 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. * Seccion de Estudios de Postgrado e Investigacion, Escuela Superior de Medicina, Instituto Politecnico Nacional, Mexico, D.F., Mexico. ‡ Subdireccion de Ensenanza e Investigacion, CMN 20 de Noviembre, ISSSTE, Mexico, D.F., Mexico. § Division de Investigacion Biomedica, Subdireccion de Ensenanza e Investigacion, CMN 20 de Noviembre, ISSSTE, Mexico, D.F., Mexico. II Unidad de Investigacion en Obesidad, Facultad de Medicina, UNAM. Clinica de Obesidad, Instituto Nacional de Ciencias Medicas y Nutricion «Salvador Zubiran». Direccion para correspondencia: Ramon M. Coral-Vazquez Seccion de Estudios de Posgrado e Investigacion, Escuela Superior de Medicina, Plan de San Luis y Diaz Miron s/n, Col. Casco de Santo Tomas, Del. Miguel Hidalgo. 11340, Mexico, D.F., Mexico. E-mail: [email protected] [email protected] Recibido: 30 de junio de 2015. Aceptado: 7 de agosto de 2015. Este articulo puede ser consultado en version completa en: http://www.medigraphic.com/rid Palabras clave: Complejo sarcoglicano, musculo estriado, tejido vascular.
Source: Investigación en Discapacidad
Language: Spanish
Subfield: Molecular Biology
Domain: Life Sciences
Open Access status: diamond
OpenAlex ID: W2346616587