Inhibitory antibodies against factor VIII C1 domain
نویسندگان
چکیده
منابع مشابه
The factor VIII C1 domain contributes to platelet binding.
Activated factor VIII (FVIIIa) forms a procoagulant complex with factor IXa on negatively charged membranes, including activated platelet surfaces. Membrane attachment involves the FVIII C2 domain; involvement of the adjacent C1 domain has not been established. Binding of recombinant FVIII C1C2 and C2 proteins to platelets was detected by flow cytometry using (1) anti-C2 monoclonal antibody ESH...
متن کاملNoncoagulation inhibitory factor VIII antibodies after induction of tolerance to factor VIII in hemophilia A patients.
We recently described tolerance induction with factor VIII/IX, cyclophosphamide, and high-dose intravenous IgG in hemophilia A or B patients with coagulation inhibitory antibodies. Circulating noninhibitory antibodies complexed with factor IX have been demonstrated in tolerant hemophilia B patients. Similar findings are now described in six tolerant hemophilia A patients. Complexes between fact...
متن کاملA macroglobulin with inhibitory activity against coagulation factor VIII.
H EMOSTATIC DISORDERS with a varying incidence of bleeding, are common in the dysproteinemias, and are characteristic of the macroglobulinemia of \\Taldenstr#{246}m These abnormalities are most frequently attributed to abnormal platelet function,1 and depression of specific clotting factors appears to be a rare occurrence. Nilehn2 includes four instances of antihemophilic globulin ( factor VIII...
متن کاملHEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY The factor VIII C1 domain contributes to platelet binding
Activated factor VIII (FVIIIa) forms a procoagulant complex with factor IXa on negatively charged membranes, including activated platelet surfaces. Membrane attachment involves the FVIII C2 domain; involvement of the adjacent C1 domain has not been established. Binding of recombinant FVIII C1C2 and C2 proteins to platelets was detected by flow cytometry using (1) anti-C2 monoclonal antibody ESH...
متن کاملA novel cause of mild/moderate hemophilia A: mutations scattered in the factor VIII C1 domain reduce factor VIII binding to von Willebrand factor.
The mechanisms responsible for the low factor VIII (fVIII) activity in the plasma of patients with mild/moderate hemophilia A are poorly understood. In such patients, we have identified a series of fVIII mutations (Ile2098Ser, Ser2119Tyr, Asn2129Ser, Arg2150His, and Pro2153Gln) clustered in the C1 domain and associated with reduced binding of fVIII to von Willebrand factor (vWf). For each patie...
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ژورنال
عنوان ژورنال: Blood
سال: 2016
ISSN: 0006-4971,1528-0020
DOI: 10.1182/blood-2016-08-727818