杜兴氏和贝克氏肌营养不良症中抗肌萎缩蛋白阳性纤维的功能意义

Functional significance of dystrophin-positive fibers in Duchenne and Becker muscular dystrophy.

作者信息

Taşdemir H A, Kotiloğlu E, Topaloğlu H, Kale G, Dinçer D P, Yalaz K, Renda Y

机构信息

Department of Pediatrics, Atutürk University Faculty of Medicine, Erzurum, Turkey.

出版信息

Pediatr Pathol Lab Med. 1996 Jul-Aug;16(4):583-90.

PMID:9025855

Abstract

In this study, the ratios of dystrophin-positive (+), partially deficient (+/-), and deficient (-) fibers were investigated immunohistochemically in 28 Duchenne muscular dystrophy (DMD) and 4 Becker muscular dystrophy (BMD) patients using Dys I (midrod), Dys II (COOH-terminal), and Dys III (NH2-terminal) antibodies. In the biopsies of DMD patients, Dys II was negative in all cases; the mean ratio of Dys I (+) fibers was 0.05%, Dys I (+/-) 1.02%, Dys III (+) 0.27%, and Dys III (+/-) 0.75%. There was no correlation between these (+) or (+/-) fibers and the severity of clinical or laboratory findings. In BMD patients, it was shown that amino and carboxyl terminals of dystrophin could be affected in addition to the midportion.

摘要

在本研究中，使用抗肌萎缩蛋白I（中间杆区域）、抗肌萎缩蛋白II（羧基末端）和抗肌萎缩蛋白III（氨基末端）抗体，通过免疫组织化学方法对28例杜氏肌营养不良症（DMD）患者和4例贝克肌营养不良症（BMD）患者的抗肌萎缩蛋白阳性（+）、部分缺陷（+/-）和缺陷（-）纤维的比例进行了研究。在DMD患者的活检样本中，抗肌萎缩蛋白II在所有病例中均为阴性；抗肌萎缩蛋白I（+）纤维的平均比例为0.05%，抗肌萎缩蛋白I（+/-）为1.02%，抗肌萎缩蛋白III（+）为0.27%，抗肌萎缩蛋白III（+/-）为0.75%。这些（+）或（+/-）纤维与临床或实验室检查结果的严重程度之间没有相关性。在BMD患者中，结果显示除了中间部分外，抗肌萎缩蛋白的氨基末端和羧基末端也可能受到影响。