Different localization of dystrophin in developing and adult human skeletal muscle
作者:
Andy Wessels,
Ieke B. Ginjaar,
Antoon F. M. Moorman,
Gert‐Jan B. Van Ommen,
期刊:
Muscle&Nerve
(WILEY Available online 1991)
卷期:
Volume 14,
issue 1
页码: 1-7
ISSN:0148-639X
年代: 1991
DOI:10.1002/mus.880140102
出版商: Wiley Subscription Services, Inc., A Wiley Company
关键词: Dystrophin localization;immunohistochemistry;human development;Duchenne muscular dystrophy;Becker muscular dystrophy
数据来源: WILEY
摘要:
AbstractDuchenne and Becker muscular dystrophy are caused by defects in dystrophin synthesis. Using affinity‐purified polyclonal anti‐dystrophin antibodies, we have studied immunohistochemically the subcellular localization of dystrophin in embryonic, fetal, and adult human skeletal muscle. In the embryonic stages dystrophin first appears in the sarcoplasm at the peripheral ends of the myotubes, immediately adjacent to the tendons, whereas in fetal stages dystrophin is found throughout the entire myofibers. In agreement with literature data, in adult muscle dystrophin expression was found to be restricted to the sarcolemma. The sarcoplasmic localization in embryonic and fetal tissue and the sarcolemmal localization of dystrophin in mature muscle suggests the accumulation of dystrophin in the cytoplasm prior to its integration into the membrane. These results increase our knowledge of the ontogenesis of dystrophin and may lead to a better understanding of the great diversity in pathological cases of Duchenne and Becker muscular dystro
点击下载:
PDF
(851KB)
返 回