Evaluation of cardiac and respiratory involvement in sarcoglycanopathies
L Politano, V Nigro, L Passamano, V Petretta… - Neuromuscular …, 2001 - Elsevier
Sarcoglycanopathies constitute a subgroup of limb-girdle recessive muscular dystrophies
due to defects in sarcoglycan complex that comprises five distinct transmembrane proteins
called α-, β-, γ-, δ-and ε-sarcoglycans. As it is well known that sarcoglycans are expressed
both in heart and in skeletal muscles and a complete deficiency in δ-sarcoglycan is the
cause of the Syrian hamster BIO. 14 cardiomyopathy, we studied cardiac and respiratory
involvement in 20 patients with sarcoglycanopathies by clinical, electrocardiographic …
due to defects in sarcoglycan complex that comprises five distinct transmembrane proteins
called α-, β-, γ-, δ-and ε-sarcoglycans. As it is well known that sarcoglycans are expressed
both in heart and in skeletal muscles and a complete deficiency in δ-sarcoglycan is the
cause of the Syrian hamster BIO. 14 cardiomyopathy, we studied cardiac and respiratory
involvement in 20 patients with sarcoglycanopathies by clinical, electrocardiographic …