Ashmun R A, Hultquist D E, Schultz J S
Am J Hematol. 1986 Dec;23(4):311-6. doi: 10.1002/ajh.2830230402.
A microspectrophotometric technique was used to measure the kinetics of methemoglobin reduction in intact, unaltered human erythrocytes. Reduction was catalyzed by endogenous NADPH-methemoglobin reductase in the presence of Nile Blue. The technique was applied to the study of erythrocytes from a female donor with decreased glucose-6-phosphate dehydrogenase (G-6-PD) activity. The individual was shown to be heterozygous for deficiency of G-6-PD. The kinetic study revealed two distinct populations of erythrocytes that were nearly equal in number. One cell population showed reduction rates between 0 and 25% of normal, whereas the second cell population displayed rates within the range seen for normal cells. Single-cell indices of cell size, cell hemoglobin content, and ratio of cell hemoglobin to cell size did not correlate with single-cell reduction rates and were not significantly different between the two populations. These results provide quantitative support for the X-inactivation hypothesis in G-6-PD deficiency.
采用显微分光光度技术测量完整、未改变的人红细胞中高铁血红蛋白还原的动力学。在尼罗蓝存在的情况下，还原反应由内源性NADPH-高铁血红蛋白还原酶催化。该技术应用于对一名葡萄糖-6-磷酸脱氢酶（G-6-PD）活性降低的女性供体的红细胞研究。结果显示该个体为G-6-PD缺乏杂合子。动力学研究揭示了数量几乎相等的两个不同红细胞群体。一个细胞群体的还原率在正常水平的0%至25%之间，而第二个细胞群体的还原率在正常细胞所见范围内。细胞大小、细胞血红蛋白含量以及细胞血红蛋白与细胞大小之比的单细胞指数与单细胞还原率无关，且两个群体之间无显著差异。这些结果为G-6-PD缺乏中的X染色体失活假说提供了定量支持。
