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本文主要对表现为溶血性贫血症的红细胞酶异常作介绍。Ⅰ.红细胞酶异常与缺乏症6-磷酸葡糖脱氢酶(G-6-PD)缺乏症、丙酮酸激酶缺乏症都是从测定红细胞酶活性发现的。与正常酶相比,它们是一种米氏(Mi-chaelis)常数(Km)高、对热不稳定、电泳泳动率不同的酶物质,尤其是 G-6-PD,因单个氨基酸被置换的就有二种,但不能排除在血红蛋白疾病中没有象地中海贫血的情况。大部分酶缺乏症是结构基因变异引起的。异常酶蛋白会以某种形式影响酶活性的表现,成为酶缺乏症。
This article focuses on the performance of hemolytic anemia erythrocyte enzyme abnormalities are introduced. Ⅰ. Erythrocyte enzyme abnormalities and deficiency 6-phosphoglucose dehydrogenase (G-6-PD) deficiency, pyruvate kinase deficiency are found from the determination of erythrocyte enzyme activity. They are a high Mi-chaelis constant (Km) enzyme enzyme that is not thermally stable and has different electrophoretic mobility ratios, especially G-6-PD, as compared to normal enzymes, as a single amino acid is replaced There are two, but can not rule out hemoglobin disease is not like thalassemia. Most enzyme deficiency is caused by structural gene variations. Abnormal enzyme protein will somehow affect the performance of enzyme activity, become enzyme deficiency.