Maldonado Martín A, Mederer Hengstl S, Gil Extremera B
An Med Interna. 1989 May;6(5):266-73.
The congenital alterations of red cell enzymes are one of the most frequent causes of red cell hemolysis. The enzyme defects have more important effects on the red cells than any other cells in the body with the same alteration. It is because red cells do not have a protein regenerating system, this meaning that they have to keep the enzyme level during the circulation time. In order to study this defect, they are classified as follows: a) defects of glycolytic pathway; b) defects of glutathionE++ pathway; c) defects of the nucleotidic metabolism. The most frequent defects are: the deficiency of: G6PD, of pyruvate kinase, of glucose phosphate isomerase and probably pyrimidine 5'-nucleotidase deficiency.
红细胞酶的先天性改变是红细胞溶血最常见的原因之一。与身体中发生相同改变的任何其他细胞相比，酶缺陷对红细胞的影响更为重要。这是因为红细胞没有蛋白质再生系统，这意味着它们必须在循环期间维持酶水平。为了研究这种缺陷，可将其分类如下：a）糖酵解途径缺陷；b）谷胱甘肽途径缺陷；c）核苷酸代谢缺陷。最常见的缺陷是：葡萄糖-6-磷酸脱氢酶、丙酮酸激酶、磷酸葡萄糖异构酶缺乏，可能还有嘧啶5'-核苷酸酶缺乏。
