[β地中海贫血中红细胞膜的分子异常]
[A molecular abnormality of the erythrocyte membrane in beta-thalassemia].
作者信息
Morle F, Morle L, Aguercif M, Alloisio N, Delaunay J
出版信息
C R Seances Soc Biol Fil. 1980;174(4):598-604.
PMID:6254622
Abstract
We present a new abnormality of the red cell membrane observed in the course of a study on beta-thalassemia. In a child suffering from a beta 0-thalassemia major, the neutral membrane bound phosphatase, instead of having Michaelis-Menten kinetics, displayed a biphasic kinetics consistent with inhibition by substrate excess. This abnormality is discussed along with other erythrocyte membrane alterations that are known to occur in beta-thalassemia.
摘要
我们报告了在β地中海贫血研究过程中观察到的一种红细胞膜新异常。在一名患有重型β0地中海贫血的儿童中，中性膜结合磷酸酶并非具有米氏动力学，而是呈现出与底物过量抑制相一致的双相动力学。本文将这种异常与已知在β地中海贫血中出现的其他红细胞膜改变一同进行讨论。
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[A molecular abnormality of the erythrocyte membrane in beta-thalassemia].[β地中海贫血中红细胞膜的分子异常]
C R Seances Soc Biol Fil. 1980;174(4):598-604.
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