InterPro : IPR002035

Name  von Willebrand factor, type A Short Name  VWF_A
Type  Domain Description  The von Willebrand factor is a large multimeric glycoprotein found in bloodplasma. Mutant forms are involved in the aetiology of bleeding disorders []. In von Willebrand factor, the type A domain (vWF) is the prototype fora protein superfamily. The vWF domain is found in various plasma proteins:complement factors B, C2, CR3 and CR4; the integrins (I-domains); collagen types VI, VII, XII and XIV; and other extracellular proteins [, , ]. Although the majority of VWA-containing proteins are extracellular, the most ancient ones present in all eukaryotes are all intracellular proteins involved in functions such as transcription, DNA repair, ribosomal and membrane transport and the proteasome. A common feature appears to be involvement in multiprotein complexes. Proteinsthat incorporate vWF domains participate in numerous biological events(e.g. cell adhesion, migration, homing, pattern formation, and signaltransduction), involving interaction with a large array of ligands []. A number of human diseases arise from mutations in VWA domains. Secondary structure prediction from 75 aligned vWF sequences has revealed a largely alternating sequence of alpha-helices and beta-strands []. The vWF domain fold is predicted to be adoubly-wound, open, twisted beta-sheet flanked by alpha-helices []. 3D structures have been determined for the I-domains of integrins CD11b(with bound magnesium) []and CD11a (with bound manganese) []. The domain adopts a classic alpha/beta Rossmann fold and contains an unusual metal ion coordination site at its surface. It has been suggested that this siterepresents a general metal ion-dependent adhesion site (MIDAS) for binding protein ligands []. The residues constituting the MIDAS motif in the CD11band CD11a I-domains are completely conserved, but the manner in which the metal ion is coordinated differs slightly [].

Sequence Features

GO Displayer


InterPro protein domain ID --> Contigs



2 Child Features

Id Name Short Name Type
IPR007198 Ssl1-like Ssl1-like Domain
IPR025861 Cobalamin biosynthesis protein CobT VWA domain CobT_VWA_dom Domain

0 Contains

24 Found In

Id Name Short Name Type
IPR012011 von Willebrand factor VWF Family
IPR012099 Midasin Midasin Family
IPR015812 Integrin beta subunit Integrin_bsu Family
IPR012170 TFIIH subunit Ssl1/p44 TFIIH_SSL1/p44 Family
IPR006538 Cobalt chelatase, CobT subunit CobT Family
IPR011360 Complement B/C2 Compl_C2_B Family
IPR004727 Calcium-activated chloride channel protein CaCC_prot Family
IPR008912 Uncharacterised protein family CoxE-like Uncharacterised_CoxE Family
IPR010607 Protein of unknown function DUF1194 DUF1194 Family
IPR011191 Uncharacterised protein family Treponema vWA Uncharacterised_Tp_vWA Family
IPR011195 Uncharacterised conserved protein UCP010256 UCP010256 Family
IPR011205 Uncharacterised conserved protein UCP015417, vWA UCP015417_vWA Family
IPR011776 Magnesium chelatase, ATPase subunit D Mg_chelatase_ATPase-dsu Family
IPR011392 Tellurite resistance, TerY Tellurite-R_TerY Family
IPR012804 Cobaltochelatase subunit, putative Cob_chelat_sub_put Family
IPR012013 Integrin beta-4 subunit Integrin_bsu-4 Family
IPR014624 Predicted 26S proteasome regulatory complex, non-ATPase subcomplex, subunit s5a, Plasmodium 26S_protsm_s5a Family
IPR015436 Integrin beta-6 subunit Integrin_bsu-6 Family
IPR015439 Integrin beta-2 subunit Integrin_bsu-2 Family
IPR016857 Uncharacterised conserved protein UCP027682, CUB, vWA UCP027682_CUB_vWA Family
IPR015442 Integrin beta-8 subunit Integrin_bsu-8 Family
IPR017360 Anthrax toxin receptor Anthrax_toxin_rcpt Family
IPR017802 VWFA-related, acidobacterial-type VWFA-rel_acidobac-type Family
IPR022440 Conserved hypothetical protein CHP03788 CHP03788 Family

0 Parent Features

7 Publications

First Author Title Year Journal Volume Pages
Bork P Shuffled domains in extracellular proteins. 1991 FEBS Lett 286 47-54
Perkins SJ The secondary structure of the von Willebrand factor type A domain in factor B of human complement by Fourier transform infrared spectroscopy. Its occurrence in collagen types VI, VII, XII and XIV, the integrins and other proteins by averaged structure predictions. 1994 J Mol Biol 238 104-19
Colombatti A Type A modules: interacting domains found in several non-fibrillar collagens and in other extracellular matrix proteins. 1993 Matrix 13 297-306
Lee JO Crystal structure of the A domain from the alpha subunit of integrin CR3 (CD11b/CD18). 1995 Cell 80 631-8
Ruggeri ZM von Willebrand factor. 1993 FASEB J 7 308-16
Edwards YJ The protein fold of the von Willebrand factor type A domain is predicted to be similar to the open twisted beta-sheet flanked by alpha-helices found in human ras-p21. 1995 FEBS Lett 358 283-6
Qu A Crystal structure of the I-domain from the CD11a/CD18 (LFA-1, alpha L beta 2) integrin. 1995 Proc Natl Acad Sci U S A 92 10277-81

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Rozanski, A., Moon, H., Brandl, H., Martín-Durán, J. M., Grohme, M., Hüttner, K., Bartscherer, K., Henry, I., & Rink, J. C.
PlanMine 3.0—improvements to a mineable resource of flatworm biology and biodiversity
Nucleic Acids Research, gky1070. doi:10.1093/nar/gky1070 (2018)