The role of membrane lipids in the survival of red cells in hereditary spherocytosis.
The role of membrane lipids in the survival of red cells in hereditary spherocytosis.
复制标题
膜脂在遗传性球形红细胞增多症中红细胞存活中的作用。
DOI：
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发表时间：
1969
影响因子：
15.9
通讯作者：
J. H. Jandl
中科院分区：
文献类型：
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作者：
R. A. Cooper;J. H. Jandl
遗传性球形红细胞增多症（HS）中的红细胞膜表面积与细胞体积的比值降低，因此呈球形。这种形态上的异常使它们易于在脾脏中积聚和破坏。虽然脾切除术可以防止HS的溶血，但红细胞缺陷，如球形、自身溶血增加、钠渗透性增加和高代谢，仍然存在。在这些表现在体外和细胞存活在体内的膜脂质的作用进行了检查。脾切除术前，尽管存在年轻细胞群，HS红细胞的胆固醇和磷脂含量降低。脾切除术后，血脂值与正常受试者的脾脏相似。然而，在脾切除术后的条件以外的HS的红细胞的脂质含量高于正常。因此，当与同样接受脾切除术的无HS患者的红细胞相比时，脾切除术后的HS细胞缺乏胆固醇和磷脂。阻塞性黄疸引起膜脂（主要是胆固醇）增加和正常红细胞渗透脆性降低。当HS红细胞输注到阻塞性黄疸患者体内时，它们也变得不那么脆弱。此外，当在阻塞性黄疸血清中孵育时，它们会获得胆固醇。这种体外膜脂的获得不会导致其葡萄糖利用率或钠流出率的变化。然而，在体内转化为不太球形的形状允许它们更好地穿过脾循环并存活更长时间。
Red cells in hereditary spherocytosis (HS) have a decreased ratio of membrane surface area to cell volume and therefore a spheroidal shape. This abnormality in shape predisposes them to pooling and destruction in the spleen. Although splenectomy prevents hemolysis in HS, the red cell defect, as manifested by spheroidicity, increased autohemolysis, excesive permeability to sodium, and hypermetabolism, persists. The role of membrane lipids in these manifestations in vitro and in cell survival in vivo was examined. Before splencetomy, and in spite of the presence of a young cell population, the cholesterol and phospholipid content of HS red cells is decreased. After splenectomy lipid values are similar to those obtained in normal subjects with spleens. However, after splenectomy for conditions other than HS the lipid content of red cells is greater than normal. Thus, when compared with the red cells of patients without HS who have also undergone splenectomy, HS cells after splenectomy are deficient in both cholesterol and phospholipid. Obstructive jaundice causes an increase in membrane lipid, primarily cholesterol, and a decrease in the osmotic fragility of normal red cells. When HS red cells are transfused into patients with obstructive jaundice they also become less osmotically fragile. Moreover, when incubated in obstructive jaundice serum, they gain cholesterol. This acquistion of membrane lipid in vitro does not result in a change in their rate of glucose utilization or sodium efflux. However, the transformation to a less spheroidal shape in vivo permits them to traverse better the splenic circulation and survive longer.