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先天性包涵体贫血患者红细胞及红细胞前体的冷冻断裂研究

Freeze-fracture study of the red cells and red cell precursors of a patient with congenital inclusion body anaemia.

作者信息

Lelkes G, Lelkes G, Hollán S R

出版信息

Haematologia (Budap). 1982;15(1):91-101.

PMID:6288522
Abstract

Comparison of thin sections and freeze fracture replicas of red cells and red cell precursors from the peripheral blood of a patient suffering from congenital inclusion body anaemia provided new morphological information on Heinz body substructure, Heinz body-membrane attachment, intracellular membrane formation, as well as on membrane fusion and orientation of intracellular membranes. It is suggested that the fusion of autophagic vacuoles with the plasma membrane may contribute to the increased haemolysis of red cells in inclusion body anaemia.

摘要

对一名先天性包涵体贫血患者外周血中的红细胞及红细胞前体的薄片和冷冻断裂复制品进行比较,提供了关于海因茨小体亚结构、海因茨小体与膜的附着、细胞内膜形成以及细胞内膜的融合和取向的新形态学信息。有人提出,自噬泡与质膜的融合可能导致包涵体贫血中红细胞溶血增加。

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