Factor VIII

Function

Factor VIII (FVIII) is a blood clotting factor. FVIII is a cofactor for factor IXa^[1] . See also Factor VIII (Hebrew).

.

(PDB code 2r7e).^[2]

Disease

Defects in the protein result in hemophilia A.

Relevance

Factor VIII is given to hemophiliacs in order to restore homestasis.

Glycosylated human Factor VIII. Residues 19-760 (cyan), 1582-2351 (magenta) complex with Ca+2 (green) and Cu+2 (orange) ions (PDB code 2r7e)

Drag the structure with the mouse to rotate

3D Structures of Factor VIII3D Structures of Factor VIII

Updated on 27-February-2023

2r7e, 3cdz, 3j2q, 4bdv, 6mf2 – hFVIII 19-760+1582-2351 – human
6mf0 – h/pFVIII
3hnb, 3hny, 3hob, 4pt6 – hFVIII light chain C2 domain 2189-2347
1d7p - hFVIII light chain C2 domain (mutant)
1cfg, 1fac - hFVIII light chain C2 domain membrane-binding peptide - NMR
7k66, 7kbt – h/pFVIII + antibody
4xzu - hFVIII light chain C2 domain + monoclonal Fab
1iqd - hFVIII light chain C2 domain (mutant) + monoclonal Fab
3j2s - hFVIII light chain 1710-2356
5k8d - hFVIII heavy chain + light chain residues 104-329
7kwo – hFVIII + VWF-XTEN
4mo3 - pFVIII light chain C2 domain – pig
7s0p - pFVIII light chain C2 domain + phosphatidylserine
4ki5 - mFVIII light chain C2 domain + monoclonal Fab – mouse

ReferencesReferences

↑ White GC 2nd, Shoemaker CB. Factor VIII gene and hemophilia A. Blood. 1989 Jan;73(1):1-12. PMID:2491949
↑ Shen BW, Spiegel PC, Chang CH, Huh JW, Lee JS, Kim J, Kim YH, Stoddard BL. The tertiary structure and domain organization of coagulation factor VIII. Blood. 2008 Feb 1;111(3):1240-7. Epub 2007 Oct 26. PMID:17965321 doi:10.1182/blood-2007-08-109918

Proteopedia Page Contributors and Editors (what is this?)Proteopedia Page Contributors and Editors (what is this?)

Alexander Berchansky, Michal Harel

[1] White GC 2nd, Shoemaker CB. Factor VIII gene and hemophilia A. Blood. 1989 Jan;73(1):1-12. PMID:2491949

[2] Shen BW, Spiegel PC, Chang CH, Huh JW, Lee JS, Kim J, Kim YH, Stoddard BL. The tertiary structure and domain organization of coagulation factor VIII. Blood. 2008 Feb 1;111(3):1240-7. Epub 2007 Oct 26. PMID:17965321 doi:10.1182/blood-2007-08-109918

[1]

[2]