Beverstock G C, Pearson P L
Acta Neurol Scand. 1982 May;65(5):413-23. doi: 10.1111/j.1600-0404.1982.tb03098.x.
There is accumulated evidence which suggests that the primary gene defect in the autosomal dominant disease of Huntington's Chorea, is given expression as a generalised membrane abnormality in peripheral tissues. Several publications claim to be able to detect a difference between HC patients and controls by means of the electron spin resonance (ESR) technique. We have examined the electron spin resonance spectra of the spin probe 4-maleimido-2,2,6,6-tetramethylpiperidinooxyl (MAL-6) when incorporated into the membranes of erythrocyte ghost cells of 22 patients with HC and 47 controls, in 2 series of controlled, blind studies and we were unable to detect any difference between HC patients and normals. We conclude that the ESR technique with the probe used is not a sufficiently reliable test for accurate differentiation of HC patients from controls and is certainly not suitable as a method for the diagnosis of potential carriers of this disease.
有越来越多的证据表明，常染色体显性疾病亨廷顿舞蹈病的主要基因缺陷，表现为外周组织的广泛性膜异常。有几篇出版物声称能够通过电子自旋共振（ESR）技术检测出亨廷顿舞蹈病患者与对照组之间的差异。我们在两项对照双盲研究中，检测了自旋探针4-马来酰亚胺-2,2,6,6-四甲基哌啶氮氧自由基（MAL-6）掺入22例亨廷顿舞蹈病患者和47例对照组的红细胞血影细胞膜后的电子自旋共振光谱，结果未能检测出患者与正常人之间的任何差异。我们得出结论，使用该探针的ESR技术并非一种足够可靠的检测方法，无法准确区分亨廷顿舞蹈病患者与对照组，当然也不适用于诊断该疾病的潜在携带者。
