Four unique transmembrane glycoproteins comprise the sarcoglycan complex in striated
muscle. The sarcoglycan complex contributes to maintenance of sarcolemma integrity. A
shared feature of four types of autosomal recessive limb girdle muscular dystrophy (LGMD)
is that mutations in a single sarcoglycan gene result in the loss of all sarcoglycans at the
sarcolemma. The mechanism of destabilization is unknown. We report here our findings of
sarcoglycan complex biosynthesis in a heterologous cell system. We demonstrate that the …