| Primary Identifier | IPR001007 | Type | Domain |
| Short Name | VWF_dom |
| description | 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 α-helices and β-strands []. The domain is named after the von Willebrand factor (VWF) type C repeat which is found in multidomain protein/multifunctional proteins involved in maintaining homeostasis [, ]. For the von Willebrand factor the duplicated VWFC domain is thought to participate in oligomerisation, but not in the initial dimerisation step []. The presence of this region in a number of other complex-forming proteins points to the possible involvement of the VWFC domain in complex formation. |
