loading...| Preferred Name |
von Willebrand Factor |
| ID |
http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C16572 |
| code |
C16572 |
| Concept_In_Subset |
http://ncicb.nci.nih.gov/xml/owl/EVS/Thesaurus.owl#C116977 |
| Contributing_Source |
CTRP |
| DEFINITION |
von Willebrand factor (2813 aa, ~309 kDa) is encoded by the human VWF gene. This protein is involved in platelet adhesion and blood coagulation. |
| DesignNote |
Human von Willebrand factor, a 270 kD multimeric plasma glycoprotein, has functional binding domains to platelet glycoprotein Ib, glycoprotein Iib/IIIa, collagen and heparin. It mediates platelet adhesion to injured vessel walls and serves as a carrier and stabiliser for coagulation factor VIII. It is expressed in endothelial cells, platelets, megakaryocytes and a number of tumors. Important in the maintenance of homeostasis, multimeric Factor VIII Related Antigen participates in platelet-vessel wall interactions by forming a non-covalent complex with coagulation factor VIII at sites of vascular injury, creating a surface to which platelets adhere. Plasma factor VIII related protein is a large glycoprotein component, synthesized by endothelial cells and megakaryocytes, that circulates in the plasma complexed with factor VIII. VWF contains three A domains, three C domains, four D domains, and one C-terminal cysteine knot-like domain. All cysteine residues of von Willebrand factor are involved in intrachain or interchain disulfide bonds. Defects in F8VWF are associated with various forms (types I-III) of von Willebrand disease (VWD). There are subtypes of type II VWD (A to H); type IIA is characterized by the absence of F8VWF multimers in plasma. |
| Display_Name |
von Willebrand Factor |
| FULL_SYN |
vWF Factor VIII Related Antigen vWFAg Coagulation Factor VIII VWF von Willebrand Factor Antigen von Willebrand Factor Factor VIII-von Willebrand Factor |
| label |
von Willebrand Factor |
| Legacy Concept Name |
von_Willebrand_Factor |
| NCI_META_CUI |
CL1770518 |
| OMIM_Number |
613160 |
| Preferred_Name |
von Willebrand Factor |
| prefixIRI |
Thesaurus:C16572 |
| prefLabel |
von Willebrand Factor |
| Semantic_Type |
Amino Acid, Peptide, or Protein |
| Swiss_Prot |
P04275 |
| subClassOf |