Platelet prothrombin converting activity in hereditary disorders of platelet function. 1986

E M Bevers, and P Comfurius, and H K Nieuwenhuis, and S Levy-Toledano, and J Enouf, and S Belluci, and J P Caen, and R F Zwaal

Prothrombinase activities of platelets have been measured in diluted platelet-rich plasma using a chromogenic substrate assay and purified coagulation factors. No abnormalities in prothrombinase activities were found for platelets from patients with storage pool disease (dense-body deficiency), grey platelet syndrome, and Glanzmann's thrombasthenia. It is concluded that neither release of dense bodies and alpha-granules nor aggregation of platelets are essential prerequisites for exposure of a procoagulant surface. Platelets from patients with Bernard-Soulier syndrome, however, have approximately 10-fold higher prothrombinase activities in the non-stimulated form than normal non-stimulated platelets. The increased procoagulant activity cannot be completely ascribed to an increase in platelet size. It is suggested that the increased prothrombinase activity reflects an increased exposure of phosphatidylserine at the outer surface of non-stimulated Bernard-Soulier platelets, earlier described by Perret et al (1983).

UI MeSH Term Description Entries
D010981 Platelet Storage Pool Deficiency Disorder characterized by a decrease or lack of platelet dense bodies in which the releasable pool of adenine nucleotides and 5HT are normally stored. Storage Pool Deficiency,Acquired Storage Pool Disease,Deficiency, Platelet Storage Pool,Deficiency, Storage Pool,Familial Platelet Storage Pool Disease,Platelet Storage Pool Deficiencies,Platelet Storage Pool Disease,Storage Pool Deficiency, Platelet,Storage Pool Platelet Disease,Deficiencies, Storage Pool,Storage Pool Deficiencies
D001791 Blood Platelet Disorders Disorders caused by abnormalities in platelet count or function. Thrombocytopathy,Blood Platelet Disorder,Disorder, Blood Platelet,Disorders, Blood Platelet,Platelet Disorder, Blood,Platelet Disorders, Blood,Thrombocytopathies
D001792 Blood Platelets Non-nucleated disk-shaped cells formed in the megakaryocyte and found in the blood of all mammals. They are mainly involved in blood coagulation. Platelets,Thrombocytes,Blood Platelet,Platelet,Platelet, Blood,Platelets, Blood,Thrombocyte
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D001606 Bernard-Soulier Syndrome A familial coagulation disorder characterized by a prolonged bleeding time, unusually large platelets, and impaired prothrombin consumption. Giant Platelet Syndrome,Deficiency of Platelet Glycoprotein 1b,Glycoprotein Ib, Platelet, Deficiency Of,Platelet Glycoprotein 1b, Deficiency of,Platelet Glycoprotein Ib Deficiency,Von Willebrand Factor Receptor Deficiency,Bernard Soulier Syndrome,Platelet Syndromes, Giant,Syndrome, Bernard-Soulier,Syndrome, Giant Platelet,Syndromes, Giant Platelet
D013915 Thrombasthenia A congenital bleeding disorder with prolonged bleeding time, absence of aggregation of platelets in response to most agents, especially ADP, and impaired or absent clot retraction. Platelet membranes are deficient in or have a defect in the glycoprotein IIb-IIIa complex (PLATELET GLYCOPROTEIN GPIIB-IIIA COMPLEX). Glanzmann Thrombasthenia,Deficiency of GP 2b 3a Complex,GP IIb-IIIa Complex, Deficiency Of,Glanzmann Thrombasthenia, Type A,Glycoprotein Complex IIb-IIIa, Deficiency Of,Platelet Fibrinogen Receptor, Deficiency of,Platelet Glycoprotein 2b-3a Deficiency,Platelet Glycoprotein IIb-IIIa Deficiency,Thrombasthenia of Glanzmann and Naegeli,GP IIb IIIa Complex, Deficiency Of,Glycoprotein Complex IIb IIIa, Deficiency Of,Platelet Glycoprotein 2b 3a Deficiency,Platelet Glycoprotein IIb IIIa Deficiency,Thrombasthenia, Glanzmann,Thrombasthenias
D013925 Thromboplastin Constituent composed of protein and phospholipid that is widely distributed in many tissues. It serves as a cofactor with factor VIIa to activate factor X in the extrinsic pathway of blood coagulation. Antigens, CD142,CD142 Antigens,Coagulation Factor III,Factor III,Tissue Factor,Tissue Thromboplastin,Blood Coagulation Factor III,Coagulin,Glomerular Procoagulant Activity,Prothrombinase,Tissue Factor Procoagulant,Urothromboplastin,Activity, Glomerular Procoagulant,Factor III, Coagulation,Procoagulant Activity, Glomerular,Procoagulant, Tissue Factor,Thromboplastin, Tissue

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