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Animal Models |
-Sarcoglycan-Deficient Hamster
From the Howard Hughes Medical Institute, Department of Physiology and Biophysics and Department of Neurology, University of Iowa College of Medicine, Iowa City, Iowa
The BIO14.6 hamster is an extensively used animal model of
autosomal recessive cardiomyopathy and muscular dystrophy.
Recently, a large deletion in the 5' end of the 
-sarcoglycan
gene was found to be the primary genetic defect in the hamster. In the
present investigation, we studied the effects of the
-sarcoglycan deletion on transcription, expression,
and function of the dystrophin-glycoprotein complex in skeletal and
cardiac muscle. We demonstrated that in striated muscle the genetic
defect leads to the complete deficiency of -sarcoglycan and a
concomitant loss of 
-, ß-, and 
-sarcoglycan. In
addition, absence of the sarcoglycan complex reduced the
expression of -dystroglycan in striated muscle fibers. These
findings indicated that the primary defect in the BIO14.6 hamster leads
to the dissociation of the dystroglycan complex from the sarcoglycan
complex and disrupted anchorage of -dystroglycan to the cell
surface. Using intravenous injection of Evans blue dye as an in
vivo tracer assay, we demonstrated that perturbation of
the dystrophin-glycoprotein complex caused extensive fiber damage in
skeletal and cardiac muscle of the BIO14.6 hamster. Based on our
results, we propose that loss of -sarcoglycan results in the
impairment of sarcolemmal integrity, finally leading to
muscular dystrophy and cardiomyopathy.
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