[HTML][HTML] Caveolin-3 null mice show a loss of caveolae, changes in the microdomain distribution of the dystrophin-glycoprotein complex, and t-tubule abnormalities
F Galbiati, JA Engelman, D Volonte, XL Zhang… - Journal of Biological …, 2001 - ASBMB
Caveolin-3, a muscle-specific caveolin-related protein, is the principal structural protein of
caveolae membrane domains in striated muscle cells. Recently, we identified a novel
autosomal dominant form of limb-girdle muscular dystrophy (LGMD-1C) in humans that is
due to mutations within the coding sequence of the human caveolin-3 gene (3p25). These
LGMD-1C mutations lead to an∼ 95% reduction in caveolin-3 protein expression, ie a
caveolin-3 deficiency. Here, we created a caveolin-3 null (CAV3−/−) mouse model, using …
caveolae membrane domains in striated muscle cells. Recently, we identified a novel
autosomal dominant form of limb-girdle muscular dystrophy (LGMD-1C) in humans that is
due to mutations within the coding sequence of the human caveolin-3 gene (3p25). These
LGMD-1C mutations lead to an∼ 95% reduction in caveolin-3 protein expression, ie a
caveolin-3 deficiency. Here, we created a caveolin-3 null (CAV3−/−) mouse model, using …