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J Biol Chem, Vol. 273, Issue 52, 34667-34670, December 25, 1998
From the Howard Hughes Medical Institute, Department of Physiology
and Biophysics, and Department of Neurology, University of Iowa
College of Medicine, Iowa City, Iowa 52242
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
sarcoglycans are glycosylated and assemble into a complex that resides
in the plasma membrane. Complex assembly was dependent on the
simultaneous synthesis of all four sarcoglycans. Mutant sarcoglycans
block complex formation and insertion of the sarcoglycans into the
plasma membrane. This constitutes the first biochemical evidence to
support the idea that the molecular defect in sarcoglycan-deficient LGMD is because of aberrant sarcoglycan complex assembly and
trafficking, which leads to the absence of the complex from the sarcolemma.
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