A patient with platelets deficient in glycoprotein VI that lack both collagen-induced aggregation and adhesion.

摘要: Molecular level studies on platelets deficient in collagen-induced aggregation provide evidence for identifying possible platelet collagen receptors. We investigated from a patient with mild bleeding time prolongation, but otherwise normal coagulation data. Her lacked and adhesion, retained release by other agonists. Labeling her 125I or 3H analysis SDS-PAGE/autoradiography showed levels of glycoproteins Ia, Ib, IIa, IIb, IIIa, IV. However, there were significantly decreased incorporations both radioactivities into 61-kD membrane glycoprotein (GP), which was identified as GPVI its mobility unreduced-reduced, two-dimensional SDS-PAGE. Sugiyama et al. (1987. Blood. 69: 1712) reported that the serum an idiopathic thrombocytopenic purpura (ITP) contained antibody against 62-kD protein. Our patient's antigen ITP antibody, demonstrating contains specific GPVI. The parents' approximately 50% amount GPVI, still had adhesion. did not bind to types I III fibrils. results suggest functions receptor.