Ginjaar I B, Virágh S, Markman M W, van Ommen G J, Moorman A F
Department of Anatomy and Embryology, Academic Medical Centre, University of Amsterdam, The Netherlands.
Microsc Res Tech. 1995 Apr 15;30(6):458-68. doi: 10.1002/jemt.1070300603.
Duchenne muscular dystrophy (DMD) is frequently associated with myocardial involvement. Dystrophin, the DMD protein, is found at the plasmamembrane of striated muscle fibers. Although dystrophin is missing in most or all muscle fibers of DMD patients, cardiac muscle is not as severely affected as skeletal muscle. Therefore it is of great importance to study the expression of dystrophin in normal cardiac muscle. We performed immunohistochemical studies and examined cardiac muscle of fetuses of 8 to 13 weeks of development on dystrophin expression. At these stages dystrophin is observed in the myocytes of the developing ventricular conduction system and in the atrial cardiomyocytes. Dystrophin was absent from the heart of a 12-week-old DMD fetus.
杜兴氏肌肉营养不良症（DMD）常伴有心肌受累。抗肌萎缩蛋白是DMD蛋白，存在于横纹肌纤维的质膜上。尽管在DMD患者的大多数或所有肌纤维中都缺失抗肌萎缩蛋白，但心肌受影响的程度不如骨骼肌严重。因此，研究抗肌萎缩蛋白在正常心肌中的表达具有重要意义。我们进行了免疫组织化学研究，并检测了8至13周发育阶段胎儿的心肌中抗肌萎缩蛋白的表达情况。在这些阶段，在发育中的心室传导系统的心肌细胞和心房心肌细胞中观察到了抗肌萎缩蛋白。在一名12周大的DMD胎儿的心脏中未检测到抗肌萎缩蛋白。
