Magnani M, Stocchi V, Canestrari F, Cucchiarini L, Stocchi O, Coppa G V, Felici L, Giorgi P L, Fornaini G
Acta Haematol. 1986;75(4):211-4. doi: 10.1159/000206127.
The levels of ATP, ADP, AMP, NADP, NADPH, NAD, NADH and reduced glutathione were determined in the red blood cells of individuals with glucose-6-phosphate dehydrogenase (G6PD) deficiency, beta-thalassemia (beta-thal) heterozygotes and in a boy carrying both mutations. The results obtained confirmed a reduced concentration of NADPH in G6PD deficiency and showed that with the combination of both diseases, the red blood cell contained practically undetectable levels of NADPH. Assays of some red blood cell enzyme activities known to be markedly influenced by cell age suggested that a younger mean red cell population is present in beta-thal/G6PD deficiency. Thus, the marked oxidative stress caused by beta-thal, that is apparently incompatible with G6PD deficiency, in fact exists, probably because of the residual activity of this enzyme in the younger red cells.
在葡萄糖-6-磷酸脱氢酶（G6PD）缺乏症患者、β地中海贫血（β-地贫）杂合子以及携带两种突变的男孩的红细胞中，测定了三磷酸腺苷（ATP）、二磷酸腺苷（ADP）、一磷酸腺苷（AMP）、烟酰胺腺嘌呤二核苷酸磷酸（NADP）、还原型烟酰胺腺嘌呤二核苷酸磷酸（NADPH）、烟酰胺腺嘌呤二核苷酸（NAD）、还原型烟酰胺腺嘌呤二核苷酸（NADH）和还原型谷胱甘肽的水平。所得结果证实G6PD缺乏症患者红细胞中NADPH浓度降低，并且表明两种疾病并存时，红细胞中的NADPH水平实际上几乎检测不到。对一些已知受细胞年龄显著影响的红细胞酶活性的测定表明，β-地贫/G6PD缺乏症患者的平均红细胞群体更年轻。因此，由β-地贫引起的明显氧化应激，这显然与G6PD缺乏症不相容，实际上是存在的，可能是因为这种酶在较年轻红细胞中的残余活性。
