Pettegrew J W, Nichols J S, Stewart R M
Ann Neurol. 1980 Oct;8(4):381-6. doi: 10.1002/ana.410080408.
Recent evidence suggests that a membrane abnormality may be present in Huntington's disease (HD), including peripheral tissues such as erythrocytes. Steady-state fluorescence spectroscopy was performed on intact erythrocytes from patients with HD and from normal age- and sex-matched controls. Fluorescent probes with known specificity for certain membrane areas were used to survey membrane dynamics; these probes included fluorescamine, which binds to primary amines on the cell surface, and both 12(9)-anthroyl stearate, 12(9)AS, and 2(9)-anthroyl stearate, 2(9)AS, which insert at different depths in the hydrocarbon core of membranes. In the intact HD erythrocyte, fluorescamine revealed decreased polarization, 12(9)AS showed increased polarization, and 2(9)AS showed no difference as compared to normal erythrocytes. These studies suggest that fluorescamine, attached to primary amines of the cell surface in intact HD erythrocytes, has increased rotational mobility whereas the anthroyl moiety of 12(9)AS in the hydrocarbon interior has decreased rotational mobility. These studies thus support the concept of a membrane abnormality in HD.
最近有证据表明,亨廷顿舞蹈病(HD)可能存在膜异常,包括红细胞等外周组织。对HD患者以及年龄和性别匹配的正常对照者的完整红细胞进行了稳态荧光光谱分析。使用对某些膜区域具有已知特异性的荧光探针来检测膜动力学;这些探针包括与细胞表面伯胺结合的荧光胺,以及12(9)-硬脂酰蒽酮(12(9)AS)和2(9)-硬脂酰蒽酮(2(9)AS),它们插入膜烃核的不同深度。在完整的HD红细胞中,荧光胺显示偏振降低,12(9)AS显示偏振增加,而2(9)AS与正常红细胞相比无差异。这些研究表明,附着在完整HD红细胞细胞表面伯胺上的荧光胺具有增加的旋转流动性,而烃内部12(9)AS的蒽酮部分具有降低的旋转流动性。因此,这些研究支持了HD中膜异常的概念。