Erythrocyte Membrane Vacuole Formation in Hereditary Spherocytosis
Erythrocyte Membrane Vacuole Formation in Hereditary Spherocytosis
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遗传性球形红细胞增多症中的红细胞膜空泡形成
DOI：
10.1111/j.1365-2141.1974.tb00449.x
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发表时间：
1974
影响因子：
6.5
通讯作者：
I. Junga
中科院分区：
文献类型：
--
作者：
S. Schrier;I. Ben;K. Bensch;M. Seeger;I. Junga
摘要遗传性球形红细胞增多症（HS）患者的红细胞中药物诱导的空泡形成受损。球状体形状本身部分解释了所观察到的空泡形成减少，但HS红细胞空泡形成中存在可由球状体解释的限制。电子显微镜显示HS中产生的空泡具有三分之一的正常直径。我们建议，减少空泡形成HS是一个结果的遗传决定异常的膜，限制其能力内陷和变形。从一个HS家族到另一个家族的空泡形成的变异性，但家族内的相对一致性，表明遗传性球形红细胞增多症包括相关的红细胞膜疾病的综合征。
Summary. There is impaired drug‐induced vacuole formation in red cells from patients with hereditary spherocytosis (HS). The spherocytic shape per se accounts in part for the decreased vacuole formation seen, but there are constraints in HS red cell vacuole formation above that explicable by spheroidicity. Electron‐microscopy indicates that the vacuoles produced in HS have one‐third normal diameter. We propose that the decreased vacuole formation in HS is a result of a genetically determined abnormality of the membrane which limits its ability to invaginate and deform. The variability in vacuole formation from one HS family to another, but relative consistency within families, suggests that hereditary spherocytosis comprises a syndrome of related erythrocyte membrane disorders.