The involvement of the sarcoglycan complex in the pathogenesis of muscular dystrophy is becoming increasingly clear. Sarcoglycan gene mutations lead to four forms of autosomal recessive limb–girdle muscular dystrophy. Recent progress has been made with the identification of novel mutations and their correlations with disease. Through this research, a better understanding the molecular pathogenesis of limb–girdle muscular dystrophy has been gained. Finally, animal models are now being used to study viral‐mediated gene transfer for the future treatment of this disease. Curr Opin Neurol 11: 443–452.© 1998 Lippincott Williams & Wilkins