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[The immunodiagnosis of thrombocyte membrane glycoprotein deficiencies: Glanzmann's thrombasthenia, Bernard-Soulier syndrome and GMP-140 protein deficiency]. 1992

O G Agafonova, and D V Vinogradov, and S G Khaspekova, and N Ia Lagutina, and T A Labinskaia, and S A Vasil'ev, and A V Mazurov

Immunological methods were developed for the diagnosis of platelet membrane glycoprotein (GP) deficiencies. The number of membrane GP on platelet surface was determined as the binding of 125I-labeled monoclonal antibodies (mAB) directed against individual platelet GP. Total amount of GP in platelet lysate was assessed by immunoblotting with specific polyclonal antibodies. Methods were applied for patients with different thrombocytopathies. Binding of mAB VM16a, directed against GP IIb-IIIa was strongly decreased in patients with Glanzmann's thrombasthenia (0.5-14.5% of normal) and binding of anti-GP Ib mAB VM16d--in patient with Bernard-Soulier syndrome (0.5% of control) indicating the deficiencies of corresponding GP. In patient with gray platelet syndrome binding of both antibodies was not decreased but even increased. It was shown by immunoblotting that platelets from the patient with gray platelet syndrome contained normal amount of GP IIa, but strongly decreased amount of GMP-140 (14.5% of control)--membrane GP of platelet--granules.

UI MeSH Term Description Entries
D007159 Immunologic Tests Immunologic techniques involved in diagnosis. Diagnosis, Immunological,Immunodiagnosis,Diagnosis, Immunologic,Immunologic Diagnosis,Immunologic Test,Immunological Tests,Tests, Immunologic,Diagnoses, Immunologic,Diagnoses, Immunological,Immunodiagnoses,Immunologic Diagnoses,Immunological Diagnoses,Immunological Diagnosis,Immunological Test,Test, Immunologic,Test, Immunological,Tests, Immunological
D010979 Platelet Function Tests Laboratory examination used to monitor and evaluate platelet function in a patient's blood. Function Test, Platelet,Function Tests, Platelet,Platelet Function Test,Test, Platelet Function,Tests, Platelet Function
D010980 Platelet Membrane Glycoproteins Surface glycoproteins on platelets which have a key role in hemostasis and thrombosis such as platelet adhesion and aggregation. Many of these are receptors. PM-GP,Platelet Glycoprotein,Platelet Membrane Glycoprotein,PM-GPs,Platelet Glycoproteins,Glycoprotein, Platelet,Glycoprotein, Platelet Membrane,Glycoproteins, Platelet,Glycoproteins, Platelet Membrane,Membrane Glycoprotein, Platelet,Membrane Glycoproteins, Platelet,PM GP
D011485 Protein Binding The process in which substances, either endogenous or exogenous, bind to proteins, peptides, enzymes, protein precursors, or allied compounds. Specific protein-binding measures are often used as assays in diagnostic assessments. Plasma Protein Binding Capacity,Binding, Protein
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
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000911 Antibodies, Monoclonal Antibodies produced by a single clone of cells. Monoclonal Antibodies,Monoclonal Antibody,Antibody, Monoclonal
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

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