| Function
Factor VIII (FVIII) is a blood clotting factor. FVIII is a cofactor for factor IXa[1] . See also Factor VIII (Hebrew).
First Ca coordination site.
Second Ca coordination site.
First Cu coordination site.
Second Cu coordination site (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.
- ↑ 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
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3D Structures of Factor VIII
Updated on 16-February-2016
factor IXa, Factor VIII (Hebrew), 2r7e, 4bdv – hFVIII – human
3hnb, 3hny, 3hob – hFVIII light chain C2 domain
1d7p - hFVIII light chain C2 domain (mutant)
1cfg, 1fac - hFVIII light chain C2 domain membrane-binding peptide - NMR
1iqd - hFVIII light chain C2 domain (mutant) + monoclonal Fab
4ki5 - mFVIII light chain C2 domain + monoclonal Fab – mouse
3j2s - hFVIII light chain
4mo3 - FVIII light chain C2 domain – pig
References
proteopedia link