Thrombasthenia with an abnormal platelet membrane glycoprotein IIb of different molecular weight.
Thrombasthenia with an abnormal platelet membrane glycoprotein IIb of different molecular weight.
复制标题
血小板无力症伴有不同分子量的异常血小板膜糖蛋白 IIb。
作者：
Stephanie M Jung;N. Yoshida;N. Aoki;K. Tanoue;H. Yamazaki;M. Moroi
我们描述了一个人与异常血小板糖蛋白（GP）IIb的不同分子量（摩尔重量），一个缺陷，区分这个病人从以前报道的血栓形成。患者为21岁女性，有轻度出血倾向;她的血小板缺乏二磷酸腺苷（ADP）聚集，胶原蛋白聚集受到严重抑制，但对利托那反应正常。她的血小板的十二烷基硫酸钠-聚丙烯酰胺凝胶电泳表明，它们含有两种类型的GPIIb分子：一种分子量异常（122 kd，未还原; 128 kd，还原），另一种分子量正常（128 kd，未还原; 118 kd，还原）。相对于正常血小板中GPIIb的量，她的血小板含有约35%的异常GPIIb和20%的正常GPIIb。对患者血小板的纤维蛋白原结合试验表明，它们含有正常量的纤维蛋白原受体的25%。患者血小板交叉免疫电泳显示主要由正常分子量GPIIb和GPIIIa组成的GPIIb/IIIa复合物形成。患者的父亲ADP聚集性降低，其血小板也含有异常和正常的GPIIb（约为正常水平的50%和纤维蛋白原受体正常数量的50%）;其母亲仅有正常的GPIIb。这些结果表明，患者具有杂合GPIIb分子，在分子水平上具有GPIIb异常。对这种异常GPIIb的研究将为GPIIb的功能及其生物合成机制提供信息。
We describe an individual with abnormal platelet glycoprotein (GP) IIb of different molecular weight (mol wt), a defect that distinguishes this patient from previously reported thrombasthenics. The patient, a 21-year-old female, has a mild bleeding tendency; her platelets lack adenosine diphosphate (ADP) aggregation and have severely suppressed collagen aggregation but a normal response to ristocetin. Sodium dodecyl sulfate-polyacrylamide gel electrophoresis of her platelets indicates that they contain two types of GPIIb molecules: one with an abnormal mol wt (122 kd, unreduced; 128 kd, reduced) and one with a normal mol wt (128 kd, unreduced; 118 kd, reduced). Relative to the amount of GPIIb in normal platelets, her platelets contain approximately 35% abnormal GPIIb and 20% normal GPIIb. Fibrinogen binding assays on the patient's platelets indicated that they contained 25% of the normal amount of fibrinogen receptors. Crossed immunoelectrophoresis of the patient's platelets demonstrated the formation of a GPIIb/IIIa complex that was mainly composed of normal mol wt GPIIb and GPIIIa. The patient's father has decreased ADP aggregability, and his platelets also contained both abnormal and normal GPIIb (about 50% of the normal level and about 50% of the normal number of fibrinogen receptors); her mother has only normal GPIIb. These results indicate that the patient has heterozygous GPIIb molecules with an abnormality of GPIIb at the molecular level. Studies on this abnormal GPIIb should provide information about the function of GPIIb and the mechanism of its biosynthesis.
影响因子：
2.9
作者：
G. Tuszynski;L. Knight;E. Kornecki;S. Srivastava
通讯作者：
G. Tuszynski;L. Knight;E. Kornecki;S. Srivastava
通过电子显微镜测定的人血小板膜糖蛋白 IIb 和 IIIa 的结构。
DOI：
--
发表时间：
1985
期刊：
The Journal of biological chemistry
影响因子：
--
作者：
Carrell,NA;Fitzgerald,LA;Steiner,B;Erickson,HP;Phillips,DR
通讯作者：
Phillips,DR