Evidence for a membrane surface defect in erythrocytes in Huntington's disease.
Evidence for a membrane surface defect in erythrocytes in Huntington's disease.
复制标题
亨廷顿病红细胞膜表面缺陷的证据。
DOI：
10.1016/0024-3205(80)90311-2
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发表时间：
1980
期刊：
影响因子：
6.1
通讯作者：
W. Markesbery
中科院分区：
文献类型：
--
作者：
D. Butterfield;P. Doorley;W. Markesbery
已经对亨廷顿病患者和正常对照的红细胞膜进行了电子自旋共振研究。在每种情况下，完整的红细胞要么未经处理，要么用膜不可渗透的酶、链霉蛋白酶、胰凝乳蛋白酶或胰蛋白酶进行蛋白水解。膜影是由未经处理和蛋白酶处理的完整细胞制备的，并用蛋白质或脂质特异性旋转探针进行旋转标记。电子自旋共振光谱的比较证实了我们之前的发现，即在未经处理的样品中，亨廷顿病中蛋白质特异性自旋标签的相关参数增加（P < 0.02），表明这种疾病中膜蛋白的物理状态发生了改变，而红细胞脂质流动性没有差异。在由蛋白酶处理的完整细胞制备的膜影中，根据自旋标记方法判断，亨廷顿病和对照中膜蛋白的物理状态没有显着差异。这些结果，连同本研究中使用的蛋白酶的已知特异性，表明亨廷顿病红细胞中的分子缺陷表现在膜蛋白的外部部分，并支持我们的假设，即亨廷顿病与普遍的细胞膜缺陷相关。
Electron spin resonance studies of erythrocyte membranes from patients with Huntington's disease and normal controls have been performed. Intact erythrocytes in each case were either untreated or subjected to proteolysis with the membrane impermeable enzymes, pronase, chymotrypsin, or trypsin. Membrane ghosts were prepared from untreated and protease-treated intact cells and spin labeled with protein- or lipid-specific spin probes. Comparison of the resulting electron spin resonance spectra confirmed our previous findings that in untreated samples the relevant parameter of the protein-specific spin label was increased in Huntington's disease (P < 0.02) suggesting an altered physical state of membrane proteins in this disorder, while no difference in erythrocyte lipid fluidity could be discerned. No significant difference in the physical state of membrane proteins in Huntington's disease and control as judged as spin labeling methods could be detemined in membrane ghosts prepared from protease-treated intact cells. These results, together with the known specificity of the proteases used in this study, suggest that a molecular defect in Huntington's disease erythrocytes is manifested in an exterior part of a membrane protein and supports our hypothesis that Huntington's disease is associated with a generalized cell membrane defect.