Berry B, Poulsen R, Yunge L, Bruneval P, Fitchett D, de Chastonay C, Gabbiani G, Hüttner I
J Mol Cell Cardiol. 1983 Aug;15(8):503-13. doi: 10.1016/0022-2828(83)90326-7.
The purpose of this study was to examine the density of intramembrane particles in the cardiac sarcolemma of normal and myopathic Syrian hamsters in a search for a morphological marker of a putative membrane defect. Hereditary cardiomyopathy in hamsters is manifested by progressive multifocal skeletal and cardiac muscle necrosis. Although pharmacological and biochemical data suggest a molecular defect in the cardiac sarcolemma, there has been no morphological observation that would substantiate this suggestion. We quantified numerical densities of intramembrane particles in freeze-fractured sarcolemmal membrane protoplasmic and extracellular faces from the left and right ventricular myocardium of female 25-day-old hamsters of the U.M.-X 7.1 cardiomyopathic line compared with sex- and age-matched randomly bred Syrian hamsters. Intramembrane particle numerical densities significantly increased above control values in cardiomyopathic hamsters for protoplasmic face (from 2188 +/- 61 to 2454 +/- 89 microns-2 in left ventricle (P less than 0.025) and from 2255 +/- 83 to 2574 +/- 56 microns-2 in right ventricle (P less than 0.001]. There was no significant difference between intramembrane particle densities of normal and cardiomyopathic hamsters for extracellular face (707 +/- 45 and 687 +/- 49 microns-2 in the left ventricle and 742 +/- 41 and 746 +/- 28 microns-2 in the right ventricle). These results implicate an increase of protoplasmic face-associated integral proteins in the sarcolemmal membrane of cardiomyopathic hamster. The significance of this finding is not known. It may represent an adaptive change related to a molecular defect in the sarcolemmal membrane of cardiomyopathic hamster.
本研究的目的是检测正常和患肌病的叙利亚仓鼠心肌肌膜中膜内颗粒的密度,以寻找假定膜缺陷的形态学标志物。仓鼠的遗传性心肌病表现为进行性多灶性骨骼肌和心肌坏死。尽管药理学和生物化学数据提示心肌肌膜存在分子缺陷,但尚无形态学观察结果能证实这一推测。我们对25日龄雌性U.M.-X 7.1心肌病系仓鼠与性别和年龄匹配的随机繁殖叙利亚仓鼠左、右心室心肌经冷冻断裂的肌膜原生质面和细胞外表面的膜内颗粒进行了数量密度定量分析。心肌病仓鼠原生质面的膜内颗粒数量密度显著高于对照值(左心室从2188±61增加到2454±89μm-2,P<0.025;右心室从2255±83增加到2574±56μm-2,P<0.001)。正常和心肌病仓鼠细胞外表面的膜内颗粒密度无显著差异(左心室分别为707±45和687±49μm-2,右心室分别为742±41和746±28μm-2)。这些结果提示心肌病仓鼠肌膜中与原生质面相关的整合蛋白增加。这一发现的意义尚不清楚。它可能代表与心肌病仓鼠肌膜分子缺陷相关的一种适应性变化。