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杜氏肌营养不良症中纽蛋白的免疫学研究。

Immunologic study of vinculin in Duchenne muscular dystrophy.

作者信息

Minetti C, Tanji K, Bonilla E

机构信息

Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, NY.

出版信息

Neurology. 1992 Sep;42(9):1751-4. doi: 10.1212/wnl.42.9.1751.

Abstract

Using immunologic techniques, we studied vinculin, a cytoskeletal protein associated with the membrane-skeleton of the muscle fiber. We examined muscle biopsies from five patients with Duchenne muscular dystrophy (DMD), two with Becker's muscular dystrophy (BMD), three normal human muscle samples, and four biopsies from disease control patients. All DMD patients showed patchy and low-intensity immunostain at the sarcolemma of most fibers and, by immunoblot analysis, the content of vinculin was 42 to 61% of control values. There was no significant vinculin deficiency in samples from patients with BMD and other disease controls. The data suggest that vinculin content is reduced only in muscle where dystrophin is absent or sparse.

摘要

我们运用免疫学技术研究了纽蛋白,一种与肌纤维膜骨架相关的细胞骨架蛋白。我们检查了5例杜氏肌营养不良症(DMD)患者、2例贝克型肌营养不良症(BMD)患者的肌肉活检样本,3份正常人类肌肉样本,以及4份疾病对照患者的活检样本。所有DMD患者的大多数肌纤维肌膜处均显示出斑片状且低强度的免疫染色,通过免疫印迹分析,纽蛋白含量为对照值的42%至61%。BMD患者和其他疾病对照患者的样本中没有明显的纽蛋白缺乏。数据表明,仅在缺乏或肌营养不良蛋白稀少的肌肉中纽蛋白含量会降低。

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