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

Idioma: Español
Referencias bibliográficas: 43
Paginas: 39-45
Archivo PDF: 340.70 Kb.

RESUMEN

Los sarcoglicanos forman parte de un grupo de proteínas transmembranales relacionadas estrechamente con un complejo mayor de proteínas asociadas con distrofina (por sus siglas en inglés, DAPC, dystrophin-associated protein complex). Inicialmente se describieron cuatro sarcoglicanos (α-, β-, γ- y δ-SG) en el sarcolema de las fibras del músculo esquelético. El complejo SG, junto con otra proteína, sarcospan (SSPN), y el subcomplejo distroglicano (DG) son parte de un andamiaje que une a la matriz extracelular con el citoesqueleto. Estas proteínas en conjunto protegen a la célula del daño mecánico durante el proceso de contracción. Adicionalmente, existen evidencias de su participación en mecanismos de transducción de señales. Mutaciones en estas proteínas son la causa de distrofias musculares de cintura 2C-F (LGMD 2C-F, limb-girdle muscular dystrophy). Existen otras isoformas de los SG, ε- y ζ-, que no se han asociado con distrofia muscular, aunque mutaciones en ε-SG pueden causar patologías como la distonía mioclónica. La deficiencia o ausencia de β-, γ- y δ-SG también se asocia con cardiopatía dilatada. En relación a esto, varios estudios han puesto de manifiesto la presencia de formas alternas del complejo SG en músculo liso y endotelio y la relevancia de estas proteínas en la fisiología vascular.

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