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小鼠中锚蛋白和血影蛋白缺陷红细胞的棘状细胞转化受损。

Impaired echinocytic transformation of ankyrin- and spectrin-deficient erythrocytes in mice.

作者信息

Reinhart W H, Sung L P, Sung K L, Bernstein S E, Chien S

机构信息

Department of Physiology, Columbia University, College of Physicians and Surgeons, New York City, New York.

出版信息

Am J Hematol. 1988 Dec;29(4):195-200. doi: 10.1002/ajh.2830290404.

DOI:10.1002/ajh.2830290404
PMID:2973228
Abstract

The membrane skeleton of the red blood cell plays an important role in the determination of cell deformability and cell shape. Under various in vitro conditions, red blood cells undergo an echinocytic or stomatocytic shape transformation. The mechanism of this fundamental process is not well understood. We have studied the red cell shape transformation in normoblastic anemia mice (nb/nb) and spherocytic anemia mice (sph/sph), which are deficient in ankyrin and spectrin, respectively. We found that both ankyrin-deficient cells (nb/nb) and spectrin-deficient cells (sph/sph) have a reduced capacity to undergo echinocytic transformation with various echinocytogenic treatments, that is, incubation with sodium salicylate (40 and 120 mM), calcium loading (50 microM A23187 + 2.2 mM Ca2+), or metabolic depletion (24 hr at 37 degrees C). These results suggest that the functional integrity of the membrane skeleton is essential for the maintenance and transformation of the red cell shape.

摘要

红细胞的膜骨架在决定细胞变形性和细胞形状方面起着重要作用。在各种体外条件下,红细胞会发生棘状红细胞或口形红细胞形状转变。这一基本过程的机制尚未完全了解。我们研究了分别缺乏锚蛋白和血影蛋白的正成红细胞性贫血小鼠(nb/nb)和球形红细胞性贫血小鼠(sph/sph)的红细胞形状转变。我们发现,无论是缺乏锚蛋白的细胞(nb/nb)还是缺乏血影蛋白的细胞(sph/sph),在接受各种致棘状红细胞生成处理时,即与水杨酸钠(40和120 mM)孵育、钙负载(50 microM A23187 + 2.2 mM Ca2+)或代谢耗竭(37摄氏度下24小时),发生棘状红细胞转变的能力均降低。这些结果表明,膜骨架的功能完整性对于维持和转变红细胞形状至关重要。

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1
Impaired echinocytic transformation of ankyrin- and spectrin-deficient erythrocytes in mice.小鼠中锚蛋白和血影蛋白缺陷红细胞的棘状细胞转化受损。
Am J Hematol. 1988 Dec;29(4):195-200. doi: 10.1002/ajh.2830290404.
2
Resistance to malaria in ankyrin and spectrin deficient mice.锚蛋白和血影蛋白缺陷小鼠对疟疾的抗性
Br J Haematol. 1991 Aug;78(4):555-60. doi: 10.1111/j.1365-2141.1991.tb04488.x.
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Splenectomy prolongs in vivo survival of erythrocytes differently in spectrin/ankyrin- and band 3-deficient hereditary spherocytosis.脾切除术对血影蛋白/锚蛋白缺陷型和带3缺陷型遗传性球形红细胞增多症患者体内红细胞存活时间的延长作用有所不同。
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Decreased content of protein 4.2 in ankyrin-deficient normoblastosis (nb/nb) mouse red blood cells: evidence for ankyrin enhancement of protein 4.2 membrane binding.锚蛋白缺陷型幼红细胞增多症(nb/nb)小鼠红细胞中蛋白质4.2含量降低:锚蛋白增强蛋白质4.2膜结合的证据。
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Echinocyte-stomatocyte transformation and shape control of human red blood cells: morphological aspects.人红细胞的棘形红细胞-口形红细胞转化及形态控制:形态学方面
Am J Hematol. 1987 Jan;24(1):1-14. doi: 10.1002/ajh.2830240102.
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Spectrin deficient inherited hemolytic anemias in the mouse: characterization by spectrin synthesis and mRNA activity in reticulocytes.小鼠中血影蛋白缺陷型遗传性溶血性贫血:通过网织红细胞中的血影蛋白合成和mRNA活性进行表征。
Cell. 1984 Jul;37(3):721-9. doi: 10.1016/0092-8674(84)90408-2.
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Deficiency of protein 4.2 in erythrocytes from a patient with a Coombs negative hemolytic anemia. Evidence for a role of protein 4.2 in stabilizing ankyrin on the membrane.一位库姆斯试验阴性溶血性贫血患者红细胞中蛋白质4.2的缺乏。蛋白质4.2在稳定膜上锚蛋白中的作用证据。
J Clin Invest. 1988 Mar;81(3):893-901. doi: 10.1172/JCI113400.
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Membrane protein phosphorylation during stomatocyte-echinocyte transformation of human erythrocytes.人红细胞口形细胞-棘形细胞转化过程中的膜蛋白磷酸化
Biochim Biophys Acta. 1986 Nov 6;862(1):1-7. doi: 10.1016/0005-2736(86)90462-1.
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Erythropoiesis in ha/ha and sph/sph mice, mutants which produce spectrin-deficient erythrocytes.在ha/ha和sph/sph小鼠(产生血影蛋白缺陷红细胞的突变体)中的红细胞生成。
Blood. 1982 Mar;59(3):646-51.
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Ankyrin and the hemolytic anemia mutation, nb, map to mouse chromosome 8: presence of the nb allele is associated with a truncated erythrocyte ankyrin.锚蛋白与溶血性贫血突变基因nb定位于小鼠8号染色体:nb等位基因的存在与截短的红细胞锚蛋白有关。
Proc Natl Acad Sci U S A. 1990 Apr;87(8):3117-21. doi: 10.1073/pnas.87.8.3117.

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