sarcoglycan Anticuerpos

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Vea tambiénsarcoglycan Antibodies incluyendo, alpha-sarcoglycan, beta-sarcoglycan, delta-sarcoglycan, epsilon-sarcoglycan, gamma-sarcoglycan, zeta-Sarcoglycan

Four sarcoglycan subunit proteins, designated α-, β-, γ- and δ-sarcoglycan, form a complex on the skeletal muscle cell surface membrane. A genetic defect in any one of these proteins causes the loss or marked decrease of the whole sarcoglycan complex, which is observed in the autosomal recessive muscular dystrophy and sarcoglycanopathy. An additional sarcogylcan, ζ-Sarcoglycan (Zeta-sarcoglycan), is a component of the vascular smooth muscle sarcoglycan complex. In muscular dystrophy, expression of zeta;-Sarcoglycan is reduced at the membrane, leading to membrane instability. This suggests that zeta;-Sarcoglycan may play a critical role in the pathogenesis of muscular dystrophy. In smooth muscle, β- and δ-sarcoglycans are associated with ε-sarcoglycan, a glycoprotein homologous to alpha;-sarcoglycan. Additionally, a complete deficiency in δ-sarcoglycan is the cause of the Syrian hamster BIO.14 cardiomyopathy