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[用于杜兴氏和贝克氏进行性肌营养不良活检诊断的抗肌营养不良蛋白单克隆抗体]

[Monoclonal antibodies to dystrophin in biopsy diagnosis of Duchenne and Becker progressive muscular dystrophies].

作者信息

Lukás Z, Foretová L, Vojtísková M, Dráber P, Hájek J

机构信息

II. patologicko-anatomický ústav LF MU, Brno.

出版信息

Cesk Patol. 1994 May;30(2):37-42.

PMID:8020113
Abstract

Immunohistochemical method detecting dystrophin in muscle biopsies was introduced and applied in 121 cases with a large scale of neuromuscular diseases. A monoclonal antibody NCL-DYS 2 (Novocastra) was used for the detection of C-terminal domain of dystrophin. Normal, i.e. sarcolemmal, localization of dystrophin was found in controls, in inactivity atrophy, neurogenic lesions and congenital myopathies. A similar situation except regenerating fibres was found in myositis and progressive muscular dystrophies different from Duchenne (DMD) and Becker (BMD) types, DMD cases showed a complete or nearly complete loss of sarcolemmal reaction product, whereas a partial loss of dystrophin in membrane was found in BMD cases as well as in transmitter females. Fibres splitting during neurogenic and myogenic lesions had dystrophin in newly produced sarcolemmal parts. Sarcolemmal immunoreactivity starting as early as in the 10th-12th week of gestation was found in human fetuses.

摘要

介绍了免疫组织化学方法检测肌肉活检中的肌营养不良蛋白,并将其应用于121例患有多种神经肌肉疾病的患者。使用单克隆抗体NCL-DYS 2(诺华卡斯达)检测肌营养不良蛋白的C末端结构域。在对照组、失用性萎缩、神经源性病变和先天性肌病中发现肌营养不良蛋白呈正常,即肌膜定位。在肌炎和不同于杜氏(DMD)和贝克(BMD)型的进行性肌营养不良症中,除了再生纤维外也发现了类似情况,DMD病例显示肌膜反应产物完全或几乎完全缺失,而在BMD病例以及携带者女性中发现肌膜中肌营养不良蛋白部分缺失。在神经源性和肌源性病变过程中分裂的纤维在新形成的肌膜部分含有肌营养不良蛋白。在人类胎儿中发现早在妊娠第10 - 12周就开始有肌膜免疫反应性。

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