An inherited and complete deficiency of diphosphoglycerate mutase was discovered in the erythrocytes of a 42-yr-old man of French origin whose blood hemoglobin concentration was 19.0 g/dl. Upon physical examination he was normal with the exception of a ruddy cyanosis. The morphology of his erythrocytes was also normal and there was no evidence of hemolysis. The erythrocyte 2,3-diphosphoglycerate level was below 3% of normal values and, as a consequence, the affinity of the cells for oxygen was increased. Diphosphoglycerate mutase activity was undetectable in erythrocytes as was that of diphosphoglycerate phosphatase. The activities of all the other erythrocyte enzymes that were tested were normal except for nomophosphoglycerate mutase which was diminished to 50% of the normal value. The levels of reduced glutathione, ATP, fructose 1,6-diphosphate, and of triose phosphates were elevated, whereas those of glucose 6-phosphate and fructose 6-phosphate were decreased. This report sheds new light on the role of diphosphoglycerate mutase in the metabolism of erythrocytes.

译文

:在一名法国血统血红蛋白浓度为19.0 g / dl的42岁男性的红细胞中发现了遗传性和完全缺乏的二磷酸甘油酸突变酶。经身体检查,除红病外,他一切正常。他的红细胞形态也正常,没有溶血的迹象。红细胞2,3-二磷酸甘油酸水平低于正常值的3%,因此,细胞对氧气的亲和力增加。与磷酸二甘油酯磷酸酶一样,在红细胞中不能检测到磷酸二甘油酯突变酶的活性。测试的所有其他红细胞酶的活性都是正常的,除了磷酸磷酸甘油酯变位酶降低到正常值的50%。还原型谷胱甘肽,ATP,果糖1,6-二磷酸和磷酸三糖的水平升高,而葡萄糖6-磷酸和果糖6-磷酸的水平降低。该报告为二磷酸甘油酸突变酶在红细胞代谢中的作用提供了新的启示。

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