@entity17325 types 2E and F are characterized by skeletal @entity425 and often @entity296 and are due to mutations in the genes encoding beta- and delta-sarcoglycan. We previously demonstrated that loss of sarcoglycans in smooth muscle leads to constrictions of the microvasculature that contributes to the cardiac phenotype. It is unclear how @entity854 affect skeletal muscle. We injected recombinant beta- or delta-sarcoglycan adenoviruses into skeletal muscles of corresponding null @entity19 . We hypothesized that the adenoviruses would not transduce vascular smooth muscle, and we would only target skeletal muscle. Indeed, sustained expression of intact sarcoglycan-sarcospan complex was noted at the sarcolemma, neuromuscular junction, myotendinous junction, and in peripheral nerve, but not in vascular smooth muscle. Gene transfer of the corresponding deleted sarcoglycan gene preserved sarcolemmal integrity, prevented @entity3986 and @entity1184 , and protected against exercised-induced damage. We conclude that @entity455 is not a primary cause of beta- and delta-sarcoglycan-deficient @entity3986 . In addition, we show successful functional rescue of entire muscles after adenovirus-mediated gene delivery. Thus, virus-mediated gene transfer of sarcoglycans to skeletal muscle in combination with pharmacological prevention of @entity296 constitute promising therapeutic strategies for @entity17325 .

Gene transfer establishes primacy of striated vs. smooth muscle sarcoglycan complex in XXXX .

@entity296 :: ('MESH:D009202', 'Disease') :: ['cardiomyopathy'] @entity455 :: ('MESH:D014652', 'Disease') :: ['vascular dysfunction'] @entity19 :: ('10090', 'Species') :: ['mice'] @entity1184 :: ('MESH:D006984', 'Disease') :: ['hypertrophy'] @entity3986 :: ('MESH:D009136', 'Disease') :: ['pathological dystrophy', 'muscular dystrophy'] @entity425 :: ('MESH:D018908', 'Disease') :: ['muscle weakness'] @entity854 :: ('MESH:D018376', 'Disease') :: ['vasculature abnormalities'] @entity17325 :: ('MESH:D049288', 'Disease') :: ['Limb-girdle muscular dystrophy', 'limb-girdle muscular dystrophies']
@entity17325 :: (MESH:D049288,Disease) :: ['limb-girdle muscular dystrophy']
