Factor VIII

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[[Image:2r7e.png|left|200px|thumb|Crystal Structure of human Factor VIII, [[2r7e]]]]
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<StructureSection load='2r7e' size='350' side='right' scene='45/451726/Cv/2' caption='Glycosylated human Factor VIII. Residues 19-760 (cyan), 1582-2351 (magenta) complex with Ca+2 (green) and Cu+2 (orange) ions (PDB code [[2r7e]]) '>
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{{STRUCTURE_2r7e| PDB=2r7e | SIZE=400| SCENE=Factor_VIII/Cv/1 |right|CAPTION=Glycosylated human Factor VIII. Residues 19-760 (grey) 1582-2351 (green) complex with Ca+2 and Cu+2 ions, [[2r7e]] }}
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== Function ==
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[[Factor VIII]] (FVIII) is a blood clotting factor. FVIII is a cofactor for [[Factor IX|factor IXa]]<ref>PMID:2491949</ref> . See also [[Factor VIII (Hebrew)]].
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<scene name='45/451726/Cv/7'>First Ca coordination site</scene>.
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<scene name='45/451726/Cv/8'>Second Ca coordination site</scene>.
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<scene name='45/451726/Cv/9'>First Cu coordination site</scene>.
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<scene name='45/451726/Cv/10'>Second Cu coordination site</scene> (PDB code [[2r7e]]).<ref>PMID:17965321</ref>
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== Disease ==
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Defects in the protein result in [[Hemophilia|hemophilia]] A.
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== Relevance ==
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Factor VIII is given to hemophiliacs in order to restore homestasis.
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[[Factor VIII]] (FVIII) is a blood clotting factor. Defects in the protein result in hemophilia A. FVIII is a cofactor for [[Factor IX|factor IXa]]. The images at the left and at the right correspond to one representative FVIII, ''i.e.'' the crystal structure of human Factor VIII ([[2r7e]]).
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===RECOMBINANT HUMAN CELLULAR COAGULATION FACTOR XIII===
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The A subunit is composed of an activation peptide and 4 domains: the β-sandwich <scene name='1f13/Chaina_domain1/1'>domain</scene>, the central core domain, the barrel 1 domain, and the
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<scene name='1f13/Fxiii_4domains_colored/1'>barrel 2 domain</scene>.-->
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{{TOC limit|limit=2}}
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<br /><br />
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</StructureSection>
== 3D Structures of Factor VIII ==
== 3D Structures of Factor VIII ==
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Updated on {{REVISIONDAY2}}-{{MONTHNAME|{{REVISIONMONTH}}}}-{{REVISIONYEAR}}
Updated on {{REVISIONDAY2}}-{{MONTHNAME|{{REVISIONMONTH}}}}-{{REVISIONYEAR}}
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[[2r7e]], [[3cdz]] – hFVIII – human<br />
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[[2r7e]], [[3cdz]], [[3j2q]], [[4bdv]], [[6mf2]] – hFVIII 19-760+1582-2351 – human<br />
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[[3hnb]], [[3hny]], [[3hob]] – hFVIII light chain C2 domain<br />
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[[6mf0]] – h/pFVIII<br />
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[[3hnb]], [[3hny]], [[3hob]], [[4pt6]] – hFVIII light chain C2 domain 2189-2347<br />
[[1d7p]] - hFVIII light chain C2 domain (mutant)<br />
[[1d7p]] - hFVIII light chain C2 domain (mutant)<br />
[[1cfg]], [[1fac]] - hFVIII light chain C2 domain membrane-binding peptide - NMR<br />
[[1cfg]], [[1fac]] - hFVIII light chain C2 domain membrane-binding peptide - NMR<br />
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[[1iqd]] - hFVIII light chain C2 domain (mutant) + monoclonal Fab<br />
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[[7k66]], [[7kbt]] – h/pFVIII + antibody<br />
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[[4xzu]] - hFVIII light chain C2 domain + monoclonal Fab <br />
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[[1iqd]] - hFVIII light chain C2 domain (mutant) + monoclonal Fab<br />
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[[3j2s]] - hFVIII light chain 1710-2356<br />
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[[5k8d]] - hFVIII heavy chain + light chain residues 104-329<br />
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[[7kwo]] – hFVIII + VWF-XTEN<br />
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[[4mo3]] - pFVIII light chain C2 domain – pig<br />
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[[7s0p]] - pFVIII light chain C2 domain + phosphatidylserine<br />
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[[4ki5]] - mFVIII light chain C2 domain + monoclonal Fab – mouse<br />
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== References ==
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<references/>
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[[Category:Topic Page]]
[[Category:Topic Page]]

Current revision

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 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

References

  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

Proteopedia Page Contributors and Editors (what is this?)

Michal Harel, Alexander Berchansky

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