Robenek Horst, Weissen-Plenz Gabriele, Severs Nicholas J
Leibniz-Institute for Arteriosclerosis Research, Department of Cell Biology and Ultrastructure Research, University of Münster, Münster, Germany.
J Cell Mol Med. 2008 Dec;12(6A):2519-21. doi: 10.1111/j.1582-4934.2008.00498.x. Epub 2008 Sep 13.
Mutations in or ablation of the gene encoding caveolin-3, a protein of muscle cell caveolae, result in forms of muscular dystrophy and cardiomyopathy. Another member of the caveolin gene family, caveolin-1, is widely considered not to be expressed in myocytes, yet ablation of the gene encoding this protein in mice also results in cardiomyopathy. By applying the high-resolution electron-microscopical imaging technique of freeze-fracture replica immunolabelling, we report here evidence that caveolin-1 is expressed in human cardiac myocytes, localized to both caveolae and non-caveolar domains in the plasma membrane. Disorders of the myocyte resulting from defects in caveolin-1 may thus arise directly, at the level of the myocyte, rather than via other cell types as previously proposed.
编码小窝蛋白-3(一种肌细胞小窝蛋白)的基因突变或该基因缺失会导致多种形式的肌肉萎缩症和心肌病。小窝蛋白基因家族的另一个成员小窝蛋白-1,普遍认为在心肌细胞中不表达,但在小鼠中该蛋白编码基因的缺失也会导致心肌病。通过应用冷冻蚀刻复型免疫标记的高分辨率电子显微镜成像技术,我们在此报告证据表明,小窝蛋白-1在人类心肌细胞中表达,定位于质膜的小窝和非小窝区域。因此,由小窝蛋白-1缺陷导致的心肌细胞紊乱可能直接发生在心肌细胞水平,而不是如先前提出的那样通过其他细胞类型。