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J. Cell Biol.,
Volume 143, Number 7, December 28, 1998 2033-2044
* Howard Hughes Medical Institute, The sarcoglycans are a complex of four
transmembrane proteins ( Division of Genetics, and § Department of Pathology, Children's Hospital and Harvard
Medical School, Boston, Massachusetts 02115
, 
, 
, and 
) which are primarily expressed in skeletal muscle and are closely associated with dystrophin and the dystroglycans in the
muscle membrane. Mutations in the sarcoglycans are
responsible for four autosomal recessive forms of muscular dystrophy. The function and the organization of
the sarcoglycan complex are unknown. We have used
coimmunoprecipitation and in vivo cross-linking techniques to analyze the sarcoglycan complex in cultured
mouse myotubes. We demonstrate that the interaction
between - and -sarcoglycan is resistant to high concentrations of SDS and -sarcoglycan is less tightly associated with other members of the complex. Cross-linking experiments show that -, -, and -sarcoglycan
are in close proximity to one another and that
-sarcoglycan can be cross-linked to the dystroglycan
complex. In addition, three of the sarcoglycans (, ,
and ) are shown to form intramolecular disulfide
bonds. These studies further our knowledge of the
structure of the sarcoglycan complex. Our proposed
model of their interactions helps to explain some of the
emerging data on the consequences of mutations in the
individual sarcoglycans, their effect on the complex,
and potentially the clinical course of muscular dystrophies.
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