von Willebrand factor

VWF
Available structures
PDB	Ortholog search: PDBe RCSB
List of PDB id codes
	1AO3, 1ATZ, 1AUQ, 1FE8, 1FNS, 1IJB, 1IJK, 1M10, 1OAK, 1U0N, 2ADF, 3GXB, 3HXO, 3HXQ, 3PPV, 3PPW, 3PPX, 3PPY, 3ZQK, 4DMU, 1UEX, 2MHP, 2MHQ, 4C29, 4C2A, 4C2B, 4NT5, 5BV8
Identifiers
Aliases	VWF, F8VWD, von Willebrand factor
External IDs	OMIM: 613160; MGI: 98941; HomoloGene: 466; GeneCards: VWF; OMA:VWF - orthologs
Gene location (Human)
Chr.	Chromosome 12 (human)
End	6,124,770 bp
Gene location (Mouse)
Chr.	Chromosome 6 (mouse)
End	125,663,642 bp
RNA expression pattern
	Top expressed in
	urethra; ; tendon of biceps brachii; ; apex of heart; ; right lung; ; upper lobe of left lung; ; pericardium; ; left ventricle; ; subcutaneous adipose tissue; ; right auricle of heart; ; lower lobe of lung;
	Top expressed in
	right lung lobe; ; external carotid artery; ; internal carotid artery; ; umbilical cord; ; tunica media of zone of aorta; ; blood; ; carotid body; ; pineal gland; ; tunica adventitia of aorta; ; sciatic nerve;
	More reference expression data
	n/a
Gene ontology
Molecular function	chaperone binding; protein homodimerization activity; collagen binding; protein N-terminus binding; immunoglobulin binding; protease binding; integrin binding; identical protein binding; protein binding; extracellular matrix structural constituent;
Cellular component	platelet alpha granule; Weibel-Palade body; extracellular region; endoplasmic reticulum; extracellular exosome; platelet alpha granule lumen; extracellular matrix; collagen-containing extracellular matrix;
Biological process	hemostasis; blood coagulation, intrinsic pathway; platelet degranulation; extracellular matrix organization; blood coagulation; cell-substrate adhesion; cell adhesion; response to wounding; protein homooligomerization; platelet activation;
	Sources:Amigo / QuickGO
Orthologs
	7450
	22371
	ENSG00000110799
	ENSMUSG00000001930
	P04275
	Q8CIZ8
	NM_000552
	NM_011708
	NP_000543
	NP_035838
	Wikidata
View/Edit Human	View/Edit Mouse

Von Willebrand factor (VWF) (German: [fɔn ˈvɪləbʁant]) is a blood glycoprotein that promotes primary hemostasis, specifically, platelet adhesion. It is deficient and/or defective in von Willebrand disease and is involved in many other diseases, including thrombotic thrombocytopenic purpura, Heyde's syndrome, and possibly hemolytic–uremic syndrome.^[5] Increased plasma levels in many cardiovascular, neoplastic, metabolic (e.g. diabetes), and connective tissue diseases are presumed to arise from adverse changes to the endothelium, and may predict an increased risk of thrombosis.^[6]

Platelet adhesion is mainly mediated via interactions with vWF, which acts as a bridge between the platelet surface receptor glycoprotein Ib (GpIb) and the exposed collagen after vascular injury. Genetic deficiencies of vWF or GpIb (Bernard-Soulier syndrome) result in bleeding disorders.^[7]

^ ^a ^b ^c GRCh38: Ensembl release 89: ENSG00000110799 – Ensembl, May 2017
^ ^a ^b ^c GRCm38: Ensembl release 89: ENSMUSG00000001930 – Ensembl, May 2017
^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^ Sadler JE (1998). "Biochemistry and genetics of von Willebrand factor". Annual Review of Biochemistry. 67: 395–424. doi:10.1146/annurev.biochem.67.1.395. PMID 9759493.
^ Shahidi M (2017). "Thrombosis and von Willebrand Factor". Thrombosis and Embolism: From Research to Clinical Practice. Advances in Experimental Medicine and Biology. Vol. 906. pp. 285–306. doi:10.1007/5584_2016_122. ISBN 978-3-319-22107-6. PMID 27628010.
^ Kumar, Vinay; Abbas, Abul K.; Aster, Jon C.; Perkins, James A., eds. (2018). Robbins basic pathology (Tenth ed.). Philadelphia, Pennsylvania: Elsevier. ISBN 978-0-323-35317-5.

[refGRCh38Ensembl-1] GRCh38: Ensembl release 89: ENSG00000110799 – Ensembl, May 2017

[refGRCm38Ensembl-2] GRCm38: Ensembl release 89: ENSMUSG00000001930 – Ensembl, May 2017

[3] "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[4] "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[Sadler-5] Sadler JE (1998). "Biochemistry and genetics of von Willebrand factor". Annual Review of Biochemistry. 67: 395–424. doi:10.1146/annurev.biochem.67.1.395. PMID 9759493.

[6] Shahidi M (2017). "Thrombosis and von Willebrand Factor". Thrombosis and Embolism: From Research to Clinical Practice. Advances in Experimental Medicine and Biology. Vol. 906. pp. 285–306. doi:10.1007/5584_2016_122. ISBN 978-3-319-22107-6. PMID 27628010.

[7] Kumar, Vinay; Abbas, Abul K.; Aster, Jon C.; Perkins, James A., eds. (2018). Robbins basic pathology (Tenth ed.). Philadelphia, Pennsylvania: Elsevier. ISBN 978-0-323-35317-5.

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