Red cell membrane sialoglycoptein β in homozygous and heterozygous 4.1(−) hereditary elliptocytosis

Red cell membrane sialoglycoptein β in homozygous and heterozygous 4.1(−) hereditary elliptocytosis
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纯合子和杂合子 4.1(−) 遗传性椭圆红细胞增多症中的红细胞膜唾液酸糖蛋白 β

DOI:
10.1016/0005-2736(85)90392-x
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发表时间:
1985
期刊:
Biochimica et Biophysica Acta
影响因子:
--
通讯作者:
J. Delaunay
J. Delaunay
中科院分区:
--
文献类型:
--
作者:
N. Alloisio;L. Morlé;D. Bachir;D. Guetarni;P. Colonna;J. Delaunay

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唾液糖蛋白β (sialglyprotein β)是一种少量的红细胞膜唾液糖蛋白,研究了纯合子和杂合子4.1(−)遗传性椭圆细胞病,这是一种以完全或部分缺失蛋白4.1为特征的遗传性椭圆细胞病。红细胞采用周期性酸- nab3h4处理。在SDS存在下进行聚丙烯酰胺凝胶电泳,荧光图显示标记的唾液糖蛋白。(i)在4.1(−)纯合子中,唾液糖蛋白β急剧下降。目前尚不确定残留物质是唾液糖蛋白β本身,还是在同一地方迁移的不同唾液糖蛋白。在长时间暴露的荧光图中,唾液糖蛋白γ(一种与唾液糖蛋白β相关的唾液糖蛋白)也被证明是减少的。在纯合子的triton -壳中,唾液糖蛋白β和γ完全缺失。(ii)在4.1(−)杂合子中,唾液糖蛋白β略有减少,而唾液糖蛋白γ则正常。这两种蛋白质在海鳖壳中提取的量似乎正常。这些观察结果进一步支持了骨膜蛋白4.1和唾液糖蛋白β之间存在相互作用的观点,这是前一种蛋白与脂质双分子层和/或其他跨膜蛋白之间相互作用的补充。
Sialoglycoprotein β, a minor sialoglycoprotein of the red cell membrane, was studied in homozygous and heterozygous 4.1 (−) hereditary elliptocytosis, a variety of hereditary elliptocytosis characterized by total or partial absence of protein 4.1. Erythrocytes were treated with the periodic acid-NaB3H4procedure. Following polyacrylamide gel electrophoresis in the presence of SDS, labelled sialoglycoproteins were revealed by fluorography. (i) In the ghosts from the 4.1(−) homozygote, sialoglycoprotein β was sharply decreased. It is not sure whether the residual material is sialoglycoprotein β itself, or a distinct sialoglycoprotein migrating in the same place. In long exposure fluorograms, sialoglycoprotein γ (a sialoglycoprotein related to sialoglycoprotein β) also turned out to be reduced. In the homozygote's Triton-shells, sialoglycoproteins β and γ appeared completely absent. (ii) In the 4.1(−) heterozygote, sialoglycoprotein β appeared slightly reduced, whereas sialoglycoprotein γ appeared normal. Both of these proteins were extracted in seemingly normal amounts in the Triton-shells. These observations bring further support to the view that there is an inteaction between skeletal membrane protein 4.1 and sialoglycoprotein β, that is additional to other interactions between the former protein and the lipid bilayer and/or other transmembrane proteins.
红细胞膜骨骼蛋白条带4.1a和b是序列相关的磷蛋白。
DOI: --
发表时间: 1982
期刊: The Journal of biological chemistry
影响因子: --
作者:
Goodman,SR;Yu,J;Whitfield,CF;Culp,EN;Posnak,EJ
通讯作者: Posnak,EJ
遗传性和获得性溶血性贫血中的红细胞膜骨骼缺陷。
DOI: --
发表时间: 1983
影响因子: 3.6
作者:
Palek,J;Lux,SE
通讯作者: Lux,SE