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遗传性球形红细胞增多症一例特殊病例中的红细胞膜蛋白磷酸化改变

Altered erythrocyte membrane protein phosphorylation in an unusual case of hereditary spherocytosis.

作者信息

Boivin P, Delaunay J, Galand C

出版信息

Scand J Haematol. 1979 Sep;23(3):251-5. doi: 10.1111/j.1600-0609.1979.tb02700.x.

Abstract

Membrane protein phosphorylation was examined in several members from a family with an unusual form of hereditary sperocytosis. After incubation of membrane ghosts with (gamma-32 P) ATP, the phosphorylation of spectrin component II was diminished both in the absence of cAMP. In the presence of this nucleotide, the phosphorylation of components IV5 and IV8 was also decreased. Along with a previously reported alteration of a membrane neutral phosphatase in this family, these abnormalities remove the present condition from the usual form of hereditary spherocytosis.

摘要

在一个患有特殊形式遗传性球形红细胞增多症的家族的几名成员中,对膜蛋白磷酸化进行了检测。在用(γ-32P)ATP孵育膜空泡后,在缺乏环磷酸腺苷(cAMP)的情况下,血影蛋白成分II的磷酸化减少。在这种核苷酸存在的情况下,成分IV5和IV8的磷酸化也降低。连同此前报道的该家族中一种膜中性磷酸酶的改变,这些异常情况使目前的病症有别于遗传性球形红细胞增多症的常见形式。

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