Pawlak A L, Rozynkowa D
Hum Genet. 1977 Dec 29;40(1):107-11. doi: 10.1007/BF00280837.
In two studied variants of G-6-PD without chronic hemolysis in probands, sensitivity of enzymes to inhibition by NADPH was decreased. Ki for NADPH was 28 micronM in Gd Lublin and 19 micronM in Gd Poznań. Susceptibility to the oxidant-induced hemolysis was described in probands, as well as in patients hemizygous for two other variants of G-6-PD with increased Ki for NADPH. It is suggested that in these cases, the oxidant-induced hemolysis is aggravated by their inability to counteract the drop in NADPH concentration with an increase in G-6-PD activity.
在先证者中两种无慢性溶血的G-6-PD研究变体中,酶对NADPH抑制的敏感性降低。在Gd Lublin中NADPH的Ki为28微摩尔,在Gd Poznań中为19微摩尔。在先证者以及另外两种NADPH的Ki增加的G-6-PD变体的半合子患者中描述了对氧化剂诱导溶血的易感性。有人提出,在这些情况下,由于它们无法通过增加G-6-PD活性来抵消NADPH浓度的下降,从而加剧了氧化剂诱导的溶血。