Efferth T, Fabry U, Glatte P, Osieka R
Medizinische Klinik IV, RWTH Aachen, Germany.
J Mol Med (Berl). 1995 Jan;73(1):47-9. doi: 10.1007/BF00203619.
Glucose-6-phosphate dehydrogenase (G6PD) deficiency belongs to the most common human disorders of metabolism. In affected patients generation of free radicals causes life-threatening hemolytic crises, for example, after consumption of certain drugs and foods or after infections. Rather than erythrocytes we analyzed mononuclear white blood cells of a patient suffering from G6PD deficiency with respect to their ability to enter apoptosis after treatment with daunorubicin, ionizing radiation, or dexamethasone. The induction of apoptosis was increased in G6PD-deficient cells compared to cells from eight normal donors. In parallel, the glutathione content of mononuclear cells from the G6PD-deficient patient was significantly decreased. While in affected patients decreased life span of erythrocytes damaged by oxidative stress has long been recognized as the mechanism underlying hemolysis, peripheral leukocytes have not received similar attention. Induction of apoptosis is a relatively complex process that has been linked to cellular glutathione content. This is the first report investigating G6PD deficiency and apoptosis.
葡萄糖-6-磷酸脱氢酶（G6PD）缺乏症是人类最常见的代谢紊乱疾病之一。在受影响的患者中，自由基的产生会引发危及生命的溶血性危机，例如在食用某些药物和食物后或感染后。我们分析了一名患有G6PD缺乏症患者的单核白细胞在用柔红霉素、电离辐射或地塞米松治疗后进入凋亡的能力，而非红细胞。与来自八名正常供体的细胞相比，G6PD缺乏症细胞中凋亡的诱导增加。同时，来自G6PD缺乏症患者的单核细胞中谷胱甘肽含量显著降低。虽然长期以来人们一直认为在受影响的患者中，氧化应激损伤的红细胞寿命缩短是溶血的潜在机制，但外周白细胞并未受到类似的关注。凋亡的诱导是一个相对复杂的过程，与细胞谷胱甘肽含量有关。这是第一份研究G6PD缺乏症与凋亡的报告。
