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8TY1

Cryo-EM structure of coagulation factor VIII bound to NB2E9

Summary for 8TY1
Entry DOI10.2210/pdb8ty1/pdb
EMDB information41710
DescriptorCoagulation factor VIII, NB2E9 light chain, NB2E9 heavy chain, ... (4 entities in total)
Functional Keywordscoagulation, inhibitor antibody, factor viii, blood clotting-inhibitor complex, blood clotting/inhibitor
Biological sourceSus scrofa (pig)
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Total number of polymer chains3
Total formula weight219419.54
Authors
Childers, K.C.,Spiegel, P.C. (deposition date: 2023-08-24, release date: 2023-09-13, Last modification date: 2024-10-23)
Primary citationChilders, 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: 38849084
DOI: 10.1016/j.jtha.2024.05.024
PDB entries with the same primary citation
Experimental method
ELECTRON MICROSCOPY (3.46 Å)
Structure validation

227111

数据于2024-11-06公开中

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