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淡黄色带帽出血大鼠的血小板膜缺陷

Platelet membrane defects in fawn hooded bleeder rats.

作者信息

Kirchmaier C M, Meyer M, Spangenberg P, Heller R, Haroske D, Breddin H K, Till U

机构信息

Center of Internal Medicine, J.W. Goethe-University, Frankfurt/Main, FRG.

出版信息

Thromb Res. 1990 Feb 1;57(3):353-60. doi: 10.1016/0049-3848(90)90251-7.

Abstract

An inbred strain of fawn hooded rats with a congenital platelet defect shows a marked bleeding tendency with prolonged bleeding time. This haemorrhagic disorder has been exclusively related to a deficiency of nucleotides in platelet dense granules. When tested in cell electrophoresis platelets from fawn hooded bleeder rats showed a significantly lower electrophoretic mobility than normal rat platelets. Subsequent studies on the platelet membrane protein pattern by high resolution two-dimensional gel electrophoresis revealed the deficiency of a membrane glycoprotein (apparent molecular mass 90.000, isoelectric point 5.6), which is detectable in normal rat platelets after surface labeling by periodate-tritiated sodium borohydride. It seems likely, that this glycoprotein defect contributes at least partially to the disorder of platelet function in fawn hooded bleeder rats.

摘要

一种具有先天性血小板缺陷的近交系淡褐毛大鼠表现出明显的出血倾向,出血时间延长。这种出血性疾病完全与血小板致密颗粒中的核苷酸缺乏有关。在细胞电泳测试中,淡褐毛出血大鼠的血小板电泳迁移率明显低于正常大鼠血小板。随后通过高分辨率二维凝胶电泳对血小板膜蛋白图谱进行的研究显示,一种膜糖蛋白(表观分子量90,000,等电点5.6)缺乏,在用高碘酸钠-氚化硼氢化钠进行表面标记后,可在正常大鼠血小板中检测到该糖蛋白。这种糖蛋白缺陷似乎至少部分导致了淡褐毛出血大鼠的血小板功能紊乱。

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