López J A
Gladstone Institute of Cardiovascular Disease, Cardiovascular Research Institute, San Francisco, CA 94141-9100.
Blood Coagul Fibrinolysis. 1994 Feb;5(1):97-119.
The GP Ib-IX complex is part of a conglomerate of polypeptides on the platelet surface that perform several key roles of central importance to the haemostatic function of platelets. When deranged, these interactions can also lead to pathological thrombosis, with potentially disastrous consequences for the organism. In this manuscript, several aspects of the structure and biology of the complex are reviewed, including the structures of its polypeptides and their relationships to other members of a phylogenetically widespread protein family, its topology on the platelet membrane and relationship with cytoskeletal components, peptide sequences involved in binding its ligands, von Willebrand factor and thrombin, its polymorphisms, its biosynthesis, and the organizations of the genes that encode its subunits.
糖蛋白Ib-IX复合物是血小板表面多肽聚集体的一部分,这些多肽对血小板的止血功能起着几个至关重要的关键作用。当这些相互作用紊乱时,也会导致病理性血栓形成,对机体可能产生灾难性后果。在本手稿中,将综述该复合物结构和生物学的几个方面,包括其多肽结构及其与系统发育广泛的蛋白质家族其他成员的关系、其在血小板膜上的拓扑结构以及与细胞骨架成分的关系、参与结合其配体血管性血友病因子和凝血酶的肽序列、其多态性、其生物合成以及编码其亚基的基因的组织。