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镰状细胞疾病中与基质相关的βs珠蛋白链的优先结合。

Preferential binding of beta s globin chains associated with stroma in sickle cell disorders.

作者信息

Bank A, Mears G, Weiss R, O'Donnell J V, Natta C

出版信息

J Clin Invest. 1974 Oct;54(4):805-9. doi: 10.1172/JCI107820.

DOI:10.1172/JCI107820
PMID:4430714
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC301620/
Abstract

Sickle cell anemia (SS) is associated with abnormalities of the red cell membrane and decreased red cell deformability. The present study assesses globin chain binding to stroma in SS, sickle cell trait (AS), and nonsickling (AA) cells. The results indicate that there is preferential binding of newly synthesized beta(S) globin to red cell stroma in SS cells and preferential binding of beta(S) to stroma compared to beta(A) in AS cells. These studies show that beta(S) globin binding to stroma accompanies the membrane abnormalities in SS and AS patients.

摘要

镰状细胞贫血(SS)与红细胞膜异常及红细胞变形性降低有关。本研究评估了SS、镰状细胞性状(AS)和非镰状细胞(AA)细胞中珠蛋白链与基质的结合情况。结果表明,在SS细胞中,新合成的β(S)珠蛋白优先与红细胞基质结合;在AS细胞中,与β(A)相比,β(S)优先与基质结合。这些研究表明,在SS和AS患者中,β(S)珠蛋白与基质的结合伴随着膜异常。

相似文献

1
Preferential binding of beta s globin chains associated with stroma in sickle cell disorders.镰状细胞疾病中与基质相关的βs珠蛋白链的优先结合。
J Clin Invest. 1974 Oct;54(4):805-9. doi: 10.1172/JCI107820.
2
Membrane-bound hemoglobin in the erythrocytes of sickle cell anemia.镰状细胞贫血患者红细胞中的膜结合血红蛋白。
J Lab Clin Med. 1983 Nov;102(5):694-8.
3
Polyamines and globin binding in sickle cell disease.镰状细胞病中的多胺与珠蛋白结合
Am J Pediatr Hematol Oncol. 1982 Spring;4(1):73-6.
4
Red cell distribution width in sickle cell disease.镰状细胞病中的红细胞分布宽度
Ann Clin Lab Sci. 1986 Jul-Aug;16(4):274-7.
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Effects of carbon dioxide and pH variations in vitro on blood respiratory functions, red blood cell volume, transmembrane pH gradients, and sickling in sickle cell anemia.体外二氧化碳和pH值变化对镰状细胞贫血患者血液呼吸功能、红细胞体积、跨膜pH梯度及镰变的影响。
J Lab Clin Med. 1984 Aug;104(2):146-59.
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High oxygen environment during pregnancy rescues sickle cell anemia mice from prenatal death.孕期高氧环境可使镰状细胞贫血小鼠免于产前死亡。
Blood Cells Mol Dis. 2008 Jul-Aug;41(1):67-72. doi: 10.1016/j.bcmd.2007.12.002. Epub 2008 Jan 22.
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Effects of a 'sickling pulse' on the calcium and potassium permeabilities of intact, sickle trait red cells [proceedings].“镰变脉冲”对具有镰状细胞性状的完整红细胞钙通透性和钾通透性的影响[会议论文集]
J Physiol. 1978 Nov;284:93P-94P.
8
Textural differences between AA and SS blood specimens as detected by image analysis.通过图像分析检测到的AA和SS血液样本之间的质地差异。
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[Dynamic study of osmotic resistance of red cells in sickle cell anemia (author's transl)].镰状细胞贫血症红细胞渗透抵抗力的动态研究(作者译)
Pathol Biol (Paris). 1976 Sep;24(7):451-5.
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betaS Chain turnover in reticulocytes of sickle trait individuals with high or low concentrations of haemoglobin S.血红蛋白S浓度高或低的镰状性状个体网织红细胞中的βS链周转情况。
Br J Haematol. 1976 Mar;32(3):365-72. doi: 10.1111/j.1365-2141.1976.tb00940.x.

引用本文的文献

1
Cytosolic protein binding to band-3 protein inhibits endocytosis of isolated human erythrocyte membranes.胞质蛋白与带3蛋白的结合抑制了分离的人红细胞膜的内吞作用。
Biochem J. 1982 Dec 1;207(3):595-8. doi: 10.1042/bj2070595.
2
Interaction of sickle cell hemoglobin with erythrocyte membranes.镰状细胞血红蛋白与红细胞膜的相互作用。
Proc Natl Acad Sci U S A. 1981 Jan;78(1):65-8. doi: 10.1073/pnas.78.1.65.
3
Concurrent sickle cell anemia and alpha-thalassemia. Effect on pathological properties of sickle erythrocytes.镰状细胞贫血与α地中海贫血并存。对镰状红细胞病理特性的影响。
J Clin Invest. 1984 Jan;73(1):116-23. doi: 10.1172/JCI111181.
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Interaction of hemoglobin with band 3: a review.血红蛋白与带3蛋白的相互作用:综述
Klin Wochenschr. 1983 Sep 1;61(17):831-7. doi: 10.1007/BF01537457.
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Interaction of alpha-thalassemia genes with each other and with HbC in an American black family.美国一个黑人家庭中α地中海贫血基因之间及其与HbC的相互作用。
Biochem Genet. 1979 Dec;17(11-12):1021-9. doi: 10.1007/BF00504343.
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Globin chain synthesis in sickle beta-thalassaemic bone marrow and reticulocytes.镰状β地中海贫血骨髓和网织红细胞中的珠蛋白链合成
J Med Genet. 1979 Aug;16(4):296-301. doi: 10.1136/jmg.16.4.296.
7
Modification of hemoglobin H disease by sickle trait.镰状性状对血红蛋白H病的修饰作用。
J Clin Invest. 1979 Oct;64(4):1024-32. doi: 10.1172/JCI109539.

本文引用的文献

1
Abnormal human haemoglobins. Separation and characterization of the alpha and beta chains by chromatography, and the determination of two new variants, hb Chesapeak and hb J (Bangkok).异常人类血红蛋白。通过色谱法分离和鉴定α链和β链,并测定两种新变体,即血红蛋白切萨皮克和血红蛋白J(曼谷)。
J Mol Biol. 1966 Aug;19(1):91-108. doi: 10.1016/s0022-2836(66)80052-9.
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Membrane alterations associated with hemoglobinopathies.与血红蛋白病相关的膜改变。
Semin Hematol. 1970 Oct;7(4):409-26.
3
Filtration characteristics of sickle cells: rates of alteration of filterability after deoxygenation and reoxygenation, and correlations with sickling and unsickling.镰状细胞的过滤特性:脱氧和复氧后过滤性的改变速率,以及与镰变和去镰变的相关性。
J Lab Clin Med. 1970 Oct;76(4):537-47.
4
Intraerythrocytic precipitations of haemoglobins S and C.血红蛋白S和C在红细胞内的沉淀
Nat New Biol. 1972 Jan 19;235(55):88-90. doi: 10.1038/newbio235088a0.
5
Increased efficiency of exogenous messenger RNA translation in a Krebs ascites cell lysate.克氏腹水癌细胞裂解物中外源信使核糖核酸翻译效率的提高。
Proc Natl Acad Sci U S A. 1972 May;69(5):1299-303. doi: 10.1073/pnas.69.5.1299.