杜氏肌营养不良
生物
肌球蛋白
基因亚型
肌营养不良
收缩(语法)
骨骼肌
ITGA7型
肌肉收缩
刺激
再生(生物学)
细胞生物学
内分泌学
内科学
基因
生物化学
遗传学
医学
作者
Carl I. Webster,L. Silberstein,Allison G. Hays,Helen M. Blau
出处
期刊:Cell
[Elsevier]
日期:1988-02-01
卷期号:52 (4): 503-513
被引量:550
标识
DOI:10.1016/0092-8674(88)90463-1
摘要
We show that Duchenne muscular dystrophy (DMD) selectively affects a subset of skeletal muscle fibers specialized for fast contraction. Muscle fiber types were characterized immunohistochemically with monoclonal antibodies that distinguish isoforms of fetal and adult-fast or adult-slow myosin heavy chain present in the same fiber. Fetal myosin expression increased with patient age and was not due to arrested development but rather to de novo synthesis, which served as a sensitive indicator of muscle regeneration. A subset of fast fibers were the first to degenerate (type Ilb). Extensive fast fiber regeneration occurred before slow fibers were affected. These results suggest that the DMD gene product has a specific function in a subpopulation of muscle fibers specialized to respond to the highest frequency of neuronal stimulation with maximal rates of contraction.
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