| Primary Identifier | IPR001846 | Type | Domain |
| Short Name | VWF_type-D |
| description | Von Willebrand factor (VWF) is a large, multimeric blood glycoproteinsynthesized in endothelial cells and megakaryocytes, that is required fornormal hemostasis. Mutant forms are involved in the most common inheritedbleeding disorder (von Willebrand disease: VWD). VWF mediates the adhesion ofplatelets to sites of vascular damage by binding to specific platelet membraneglycoproteins and to constituents of exposed connective tissue. It is alsoessential for the transport of the blood clotting factor VIII [, ].VWF is a large multidomain protein. The type D domain (VWFD) is not onlyrequired for blood clotting factor VIII binding but also for normalmultimerization of VWF [, ]. The interaction between blood clotting factorVIII and VWF is necessary for normal survival of blood clotting factor VIII inblood circulation. The VWFD domain is a highly structured region, in which thefirst conserved Cys has been found to form a disulfide bridge with the secondconserved one [, ].The VWFD domain can occur in association with a lot of different domains likevitellogenin, VWFC, VWFA, and ZP.Proteins with a VWFD domain are listed below:Mammalian von Willebrand factor (VWF), a multifunctional protein involvedin maintaining homeostasis. It consists of 4 VWFD domains (D1-4), 3 VWFA domains,3 VWFB domains, 2 VWFC domains, an X domain and a C-terminal cystine knot [].There might be a third VWFC domain within the type B domain region []. The structure of the VWF D3 domain has been revealed []. Mammalian zonadhesin, which binds in a species-specific manner to the zonapellucida of the egg.Mammalian bone morphogenetic protein-binding (BMP-binding) endothelialregulator protein.Mammalian alpha-tectorin, which is one of the major non-collagenouscomponents of the tectorial membrane.Mammalian mucins, glycoproteins that are major constituents of theglycocalyx that covers mucosal epithelium.Mammalian vitellogenin, a major lipoprotein in many oviparous animals,which is a precursor of a lipid-binding product named as lipovitellin.This entry represents the VWFD domain. |
