8TY1
Cryo-EM structure of coagulation factor VIII bound to NB2E9
Summary for 8TY1
| Entry DOI | 10.2210/pdb8ty1/pdb |
| EMDB information | 41710 |
| Descriptor | Coagulation factor VIII, NB2E9 light chain, NB2E9 heavy chain, ... (4 entities in total) |
| Functional Keywords | coagulation, inhibitor antibody, factor viii, blood clotting-inhibitor complex, blood clotting/inhibitor |
| Biological source | Sus scrofa (pig) More |
| Total number of polymer chains | 3 |
| Total formula weight | 219419.54 |
| Authors | Childers, K.C.,Spiegel, P.C. (deposition date: 2023-08-24, release date: 2023-09-13, Last modification date: 2025-06-04) |
| Primary citation | Childers, K.C.,Cowper, B.,Vaughan, J.D.,McGill, J.R.,Davulcu, O.,Lollar, P.,Doering, C.B.,Coxon, C.H.,Spiegel Jr., P.C. Structural basis for inhibition of coagulation factor VIII reveals a shared antigenic hotspot on the C1 domain. J.Thromb.Haemost., 22:2449-2459, 2024 Cited by PubMed Abstract: Hemophilia A arises from dysfunctional or deficient coagulation factor (F)VIII and leads to inefficient fibrin clot formation and uncontrolled bleeding events. The development of antibody inhibitors is a clinical complication in hemophilia A patients receiving FVIII replacement therapy. LE2E9 is an anti-C1 domain inhibitor previously isolated from a mild/moderate hemophilia A patient and disrupts FVIII interactions with von Willebrand factor and FIXa, though the intermolecular contacts that underpin LE2E9-mediated FVIII neutralization are undefined. PubMed: 38849084DOI: 10.1016/j.jtha.2024.05.024 PDB entries with the same primary citation |
| Experimental method | ELECTRON MICROSCOPY (3.46 Å) |
Structure validation
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