Visualization of an N-terminal fragment of von Willebrand factor in complex with factor VIII

Blood

Volumn 126, Issue 8, 2015, Pages 939-942

  Yee, Andrew ^a   Oleskie, Austin N ^a   Dosey, Anne M ^a   Kretz, Colin A ^a   Gildersleeve, Robert D ^a   Dutta, Somnath ^a   Su, Min ^a   Ginsburg, David ^a,b   Skiniotis, Georgios ^a,b  

^a UNIVERSITY OF MICHIGAN   (United States)

^b UNIVERSITY OF MICHIGAN MEDICAL SCHOOL   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

BLOOD CLOTTING FACTOR 8; DIMER; MONOMER; VON WILLEBRAND FACTOR; PEPTIDE FRAGMENT;

AMINO TERMINAL SEQUENCE; ARTICLE; ELECTRON MICROSCOPY; MODEL; PLASTICITY; PRIORITY JOURNAL; PROTEIN ANALYSIS; PROTEIN INTERACTION; PROTEIN STRUCTURE; THREE DIMENSIONAL IMAGING; CHEMICAL STRUCTURE; CHEMISTRY; HUMAN; METABOLISM; PROTEIN QUATERNARY STRUCTURE;

FACTOR VIII; HUMANS; MICROSCOPY, ELECTRON; MODELS, MOLECULAR; PEPTIDE FRAGMENTS; PROTEIN STRUCTURE, QUATERNARY; VON WILLEBRAND FACTOR;

EID: 84940100034     PISSN: 00064971     EISSN: 15280020     Source Type: Journal    
DOI: 10.1182/blood-2015-04-641696     Document Type: Article

References (17)

1. Lollar P, Hill-Eubanks DC, Parker CG. Association of the factor VIII light chain with von Willebrand factor. J Biol Chem. 1988;263(21):10451-10455.
2. Lillicrap D. von Willebrand disease: advances in pathogenetic understanding, diagnosis, and therapy. Blood. 2013;122(23):3735-3740.
3. Yee A, Kretz CA. Von Willebrand factor: form for function. Semin Thromb Hemost. 2014;40(1):17-27.
4. Purvis AR, Gross J, Dang LT, et al. Two Cys residues essential for von Willebrand factor multimer assembly in the Golgi. Proc Natl Acad Sci USA. 2007;104(40):15647-15652.
5. Bendetowicz AV, Morris JA, Wise RJ, Gilbert GE, Kaufman RJ. Binding of factor VIII to von willebrand factor is enabled by cleavage of the von Willebrand factor propeptide and enhanced by formation of disulfide-linked multimers. Blood. 1998;92(2):529-538.
6. Hilbert L, Nurden P, Caron C, et al INSERM Network on Molecular Abnormalities in von Willebrand Disease. Type 2N von Willebrand disease due to compound heterozygosity for R854Q and a novel R763G mutation at the cleavage site of von Willebrand factor propeptide. Thromb Haemost. 2006;96(3):290-294.
7. Yee A, Gildersleeve RD, Gu S, et al. A von Willebrand factor fragment containing the D′D3 domains is sufficient to stabilize coagulation factor VIII in mice. Blood. 2014;124(3):445-452.
8. Foster PA, Fulcher CA, Marti T, Titani K, Zimmerman TS. A major factor VIII binding domain resides within the amino-terminal 272 amino acid residues of von Willebrand factor. J Biol Chem. 1987;262(18):8443-8446.
9. Shiltagh N, Kirkpatrick J, Cabrita LD, et al. Solution structure of the major factor VIII binding region on von Willebrand factor. Blood. 2014;123(26):4143-4151.
10. Huang RH, Wang Y, Roth R, et al. Assembly of Weibel-Palade body-like tubules from N-terminal domains of von Willebrand factor. Proc Natl Acad Sci USA. 2008;105(2):482-487.
11. Zhou YF, Eng ET, Nishida N, Lu C, Walz T, Springer TA. A pH-regulated dimeric bouquet in the structure of von Willebrand factor. EMBO J. 2011;30(19):4098-4111.
12. Miao HZ, Sirachainan N, Palmer L, et al. Bioengineering of coagulation factor VIII for improved secretion. Blood. 2004;103(9):3412-3419.
13. Wise RJ, Dorner AJ, Krane M, Pittman DD, Kaufman RJ. The role of von Willebrand factor multimers and propeptide cleavage in binding and stabilization of factor VIII. J Biol Chem. 1991;266(32):21948-21955.
14. Shen BW, Spiegel PC, Chang CH, et al. The tertiary structure and domain organization of coagulation factor VIII. Blood. 2008;111(3):1240-1247.
15. Saenko EL, Scandella D. The acidic region of the factor VIII light chain and the C2 domain together form the high affinity binding site for von willebrand factor. JBiolChem.1997;272(29):18007-18014.
16. Liu ML, Shen BW, Nakaya S, et al. Hemophilic factor VIII C1- and C2-domain missense mutations and their modeling to the 1.5-angstrom human C2-domain crystal structure. Blood. 2000;96(3):979-987.
17. Ngo JC, Huang M, Roth DA, Furie BC, Furie B. Crystal structure of human factor VIII: implications for the formation of the factor IXa-factor VIIIa complex. Structure. 2008;16(4):597-606.