Protein Domain : von Willebrand factor, type D domain IPR001846

Type	Domain
Description	Von Willebrand factor (VWF) is a large, multimeric blood glycoprotein synthesized in endothelial cells and megakaryocytes, that is required fornormal hemostasis. Mutant forms are involved in the most common inherited bleeding disorder (von Willebrand disease: VWD). VWF mediates the adhesion ofplatelets to sites of vascular damage by binding to specific platelet membrane glycoproteins 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 only required 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 in blood circulation. The VWFD domain is a highly structured region, in which thefirst conserved Cys has been found to form a disulfide bridge with the second conserved one [, ].The VWFD domain can occur in association with a lot of different domains like vitellogenin, VWFC, VWFA, and ZP.Proteins with a VWFD domain are listed below:Mammalian von Willebrand factor (VWF), a multifunctional protein involved in 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 zona pellucida of the egg.Mammalian bone morphogenetic protein-binding (BMP-binding) endothelial regulator protein.Mammalian alpha-tectorin, which is one of the major non-collagenous components of the tectorial membrane.Mammalian mucins, glycoproteins that are major constituents of the glycocalyx 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.
Short Name	VWF_type-D