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1 Division of Cardiology, Department of Medicine, and 2 Department of Neurosciences, University of California, San Diego, La Jolla, California 92093-0613
A mutation in the 
-sarcoglycan (SG) gene with absence
of -SG protein in the heart has been identified in the BIO14.6
cardiomyopathic (CM) hamster, but how the defective gene leads to
myocardial degeneration and dysfunction is unknown. We correlated left
ventricular (LV) function with increased sarcolemmal membrane
permeability and investigated the LV distribution of the
dystrophin-dystroglycan complex in BIO14.6 CM hamsters. On
echocardiography at 5 wk of age, the CM hamsters showed a mildly
enlarged diastolic dimension (LVDD) with decreased LV percent
fractional shortening (%FS), and at 9 wk further enlargement of LVDD
with reduction of %FS was observed. The percent area of myocardium
exhibiting increased membrane permeability or membrane rupture,
assessed by Evans blue dye (EBD) staining and wheat germ agglutinin,
was greater at 9 than at 5 wk. In areas not stained by EBD,
immunostaining of dystrophin was detected in CM hamsters at sarcolemma
and T tubules, as expected, but it was also abnormally expressed at the
intercalated discs; in addition, the expression of 
-dystroglycan was
significantly reduced compared with control hearts. As previously
described, 
-SG was completely deficient in CM hearts compared with
control hearts. In myocardial areas showing increased sarcolemmal
permeability, neither dystrophin nor -dystroglycan could be
identified by immunolabeling. Thus, together with the known loss of
-SG and other SGs, abnormal distribution of dystrophin and reduction
of -dystroglycan are associated with increased sarcolemmal
permeability followed by cell rupture, which correlates with early
progressive cardiac dysfunction in the BIO14.6 CM hamster.
-sarcoglycan; dystrophin-dystroglycan complex; heart failure; Evans blue dye
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