[Immunochemical study of protein anomalies in platelets of patients with Glanzmann's thrombasthenia and Bernard-Soulier syndrome].
Crossed immunoelectrophoresis of "Triton X-100" solubilized proteins from (125I) iodinated normal and abnormal (Bernard-Soulier and thrombasthenic) platelets was performed using rabbit antibodies raised against whole, normal platelets. In Bernard-Soulier platelets neither of the two surface oriented forms of GP I, glycocalicin and glycoprotein Ib (band 13) were detected. In two patients with type I thrombasthenia, the most prominent precipitate (No. 16) and platelet fibrinogen (No 24) were not detected whereas in one patient with type II thromblasthenia, fibrinogen was normally detected and the amount of protein 16 was around 15% of the normal level. The alloantibody IgG L..., able to induce a "thrombasthenic-like" reactivity in normal human platelets, has been shown to be directed against protein 16.
D000818:Animals; D001791:Blood Platelet Disorders / Q000097:blood* / Q000276:immunology; D001792:Blood Platelets / Q000032:analysis* / Q000276:immunology; D001798:Blood Proteins / Q000032:analysis*; D005340:Fibrinogen / Q000032:analysis; D006023:Glycoproteins / Q000032:analysis; D006801:Humans; D007123:Immunoelectrophoresis, Two-Dimensional / Q000379:methods; D007074:Immunoglobulin G / Q000276:immunology; D007518:Isoantibodies / Q000276:immunology; D010973:Platelet Adhesiveness; D010974:Platelet Aggregation; D011817:Rabbits
D001798:Blood Proteins; D006023:Glycoproteins; D007074:Immunoglobulin G; D007518:Isoantibodies; D005340:Fibrinogen
Etude immunochimique des anomalies protéiques des plaquettes de sujets atteints de thrombasthénie de Glanzmann et de syndrome de Bernard-Soulier.