[The immunodiagnosis of thrombocyte membrane glycoprotein deficiencies: Glanzmann's thrombasthenia, Bernard-Soulier syndrome and GMP-140 protein deficiency].
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.
Agafonova
O G
OG
Vinogradov
D V
DV
Khaspekova
S G
SG
Lagutina
N Ia
NIa
Labinskaia
T A
TA
Vasil'ev
S A
SA
Mazurov
A V
AV
D000911:Antibodies, Monoclonal; D001606:Bernard-Soulier Syndrome / Q000097:blood / Q000175:diagnosis*; D001792:Blood Platelets / Q000276:immunology; D005260:Female; D006801:Humans; D007159:Immunologic Tests / Q000379:methods; D019007:P-Selectin; D010979:Platelet Function Tests; D010980:Platelet Membrane Glycoproteins / Q000032:analysis* / Q000276:immunology; D011485:Protein Binding; D013915:Thrombasthenia / Q000097:blood / Q000175:diagnosis*
D000911:Antibodies, Monoclonal; D019007:P-Selectin; D010980:Platelet Membrane Glycoproteins
Immunodiagnostika defitsitov membrannykh glikoproteinov trombotsitov: trombastenii Glantsmanna, sindroma Bernara--Sul'e i defitsita belka GMP-140.