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无症状性贝克肌营养不良症:抗肌萎缩蛋白及抗肌萎缩蛋白相关蛋白的表达

Asymptomatic Becker muscular dystrophy: expression of dystrophin and dystrophin-related protein.

作者信息

Tachi N, Wakai S, Watanabe Y, Ohya K, Chiba S

机构信息

Department of Pediatrics, Sapporo Medical College, Japan.

出版信息

Pediatr Neurol. 1993 May-Jun;9(3):207-9. doi: 10.1016/0887-8994(93)90086-r.

Abstract

The expression of dystrophin and a dystrophin-related protein in muscle from 3 asymptomatic Becker muscular dystrophy patients is reported. Histology demonstrated moderate variation in fiber size with clustered basophilic regenerating fibers. Immunostaining for dystrophin revealed a patchy appearance on most fibers, but never on clustered regenerating fibers. Those regenerating fibers did, however, have positive immunoreactivity to a dystrophin-related protein. It is speculated that overexpression of the dystrophin-related protein in regenerating muscle fibers may contribute to the slow progression of muscle weakness or atrophy.

摘要

报告了3例无症状贝克型肌营养不良症患者肌肉中抗肌萎缩蛋白及一种抗肌萎缩蛋白相关蛋白的表达情况。组织学检查显示肌纤维大小存在中度差异,有成群的嗜碱性再生纤维。抗肌萎缩蛋白免疫染色显示大多数纤维呈斑片状外观,但成群的再生纤维上从未出现。然而,那些再生纤维对一种抗肌萎缩蛋白相关蛋白有阳性免疫反应性。据推测,再生肌纤维中抗肌萎缩蛋白相关蛋白的过表达可能导致肌无力或萎缩进展缓慢。

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