2R7E: Crystal Structure Analysis Of Coagulation Factor Viii

Citation:
Abstract
Factor VIII (fVIII) is a serum protein in the coagulation cascade that nucleates the assembly of a membrane-bound protease complex on the surface of activated platelets at the site of a vascular injury. Hemophilia A is caused by a variety of mutations in the factor VIII gene and typically requires replacement therapy with purified protein. We have determined the structure of a fully active, recombinant form of factor VIII (r-fVIII), which consists of a heterodimer of peptides, respectively containing the A1-A2 and A3-C1-C2 domains. The structure permits unambiguous modeling of the relative orientations of the 5 domains of r-fVIII. Comparison of the structures of fVIII, fV, and ceruloplasmin indicates that the location of bound metal ions and of glycosylation, both of which are critical for domain stabilization and association, overlap at some positions but have diverged at others.
PDB ID: 2R7EDownload
MMDB ID: 63781
PDB Deposition Date: 2007/9/7
Updated in MMDB: 2011/05
Experimental Method:
x-ray diffraction
Resolution: 3.7  Å
Source Organism:
Similar Structures:
Biological Unit for 2R7E: dimeric; determined by author and by software (PISA)
Molecular Components in 2R7E
Label Count Molecule
Proteins (2 molecules)
1
Coagulation Factor Viii(Gene symbol: F8)
Molecule annotation
1
Coagulation Factor Viii(Gene symbol: F8)
Molecule annotation
Chemicals (18 molecules)
1
6
2
3
3
4
4
3
5
2
* Click molecule labels to explore molecular sequence information.

Citing MMDB
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