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1IQD

Human Factor VIII C2 Domain complexed to human monoclonal BO2C11 Fab.

Summary for 1IQD
Entry DOI10.2210/pdb1iqd/pdb
Related1D7P
DescriptorHUMAN MONOCLONAL BO2C11 FAB LIGHT CHAIN, HUMAN MONOCLONAL BO2C11 FAB HEAVY CHAIN, HUMAN FACTOR VIII, ... (4 entities in total)
Functional Keywordsfactor viii, c2 domain, antibody, blood coagulation, inhibitor, bo2c11, immune system-blood clotting complex, immune system/blood clotting
Biological sourceHomo sapiens (human)
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Cellular locationSecreted, extracellular space: P00451
Total number of polymer chains3
Total formula weight63403.13
Authors
Spiegel Jr., P.C.,Jacquemin, M.,Saint-Remy, J.M.,Stoddard, B.L.,Pratt, K.P. (deposition date: 2001-07-21, release date: 2001-08-15, Last modification date: 2024-10-30)
Primary citationSpiegel Jr., P.C.,Jacquemin, M.,Saint-Remy, J.M.,Stoddard, B.L.,Pratt, K.P.
Structure of a factor VIII C2 domain-immunoglobulin G4kappa Fab complex: identification of an inhibitory antibody epitope on the surface of factor VIII.
Blood, 98:13-19, 2001
Cited by
PubMed Abstract: The development of an immune response to infused factor VIII is a complication affecting many patients with hemophilia A. Inhibitor antibodies bind to antigenic determinants on the factor VIII molecule and block its procoagulant activity. A patient-derived inhibitory immunoglobulin G4kappa antibody (BO2C11) produced by an immortalized memory B-lymphocyte cell line interferes with the binding of factor VIII to phospholipid surfaces and to von Willebrand factor. The structure of a Fab fragment derived from this antibody complexed with the factor VIII C2 domain was determined at 2.0 A resolution. The Fab interacts with solvent-exposed basic and hydrophobic side chains that form a membrane-association surface of factor VIII. This atomic resolution structure suggests a variety of amino acid substitutions in the C2 domain of factor VIII that might prevent the binding of anti-C2 inhibitor antibodies without significantly compromising the procoagulant functions of factor VIII.
PubMed: 11418455
DOI: 10.1182/blood.V98.1.13
PDB entries with the same primary citation
Experimental method
X-RAY DIFFRACTION (2 Å)
Structure validation

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