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HUMAN:VWF

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Species (Taxon ID) Homo sapiens (Human). (9606)
Gene Name(s) VWF (synonyms: F8VWF)
Protein Name(s) von Willebrand factor

vWF von Willebrand antigen 2 von Willebrand antigen II

External Links
UniProt P04275
EMBL X04385
M25865
M25828
M25829
M25830
M25831
M25832
M25833
M25834
M25835
M25836
M25837
M25838
M25839
M25840
M25841
M25842
M25843
M25844
M25845
M25846
M25847
M25848
M25849
M25850
M25851
M25852
M25853
M25854
M25855
M25856
M25857
M25858
M25859
M25860
M25861
M25862
M25863
M25864
AC005845
AC005846
AC005904
CH471116
BC022258
X04146
X06828
X06829
M17588
M10321
M60675
U81237
K03028
X02672
M16946
M16945
CCDS CCDS8539.1
PIR A34480
RefSeq NP_000543.2
UniGene Hs.440848
PDB 1AO3
1ATZ
1AUQ
1FE8
1FNS
1IJB
1IJK
1M10
1OAK
1SQ0
1U0N
1UEX
2ADF
2MHP
2MHQ
3GXB
3HXO
3HXQ
3PPV
3PPW
3PPX
3PPY
3ZQK
4C29
4C2A
4C2B
4DMU
4NT5
PDBsum 1AO3
1ATZ
1AUQ
1FE8
1FNS
1IJB
1IJK
1M10
1OAK
1SQ0
1U0N
1UEX
2ADF
2MHP
2MHQ
3GXB
3HXO
3HXQ
3PPV
3PPW
3PPX
3PPY
3ZQK
4C29
4C2A
4C2B
4DMU
4NT5
ProteinModelPortal P04275
SMR P04275
BioGrid 113289
DIP DIP-29667N
IntAct P04275
MINT MINT-244925
STRING 9606.ENSP00000261405
ChEMBL CHEMBL2021748
DrugBank DB00025
MEROPS I08.950
PhosphoSite P04275
UniCarbKB P04275
DMDM 317373549
PaxDb P04275
PRIDE P04275
Ensembl ENST00000261405
GeneID 7450
KEGG hsa:7450
UCSC uc001qnn.1
uc001qno.1
CTD 7450
GeneCards GC12M006058
GeneReviews VWF
H-InvDB HIX0010356
HIX0171640
HGNC HGNC:12726
HPA CAB001694
HPA001815
HPA002082
MIM 193400
277480
613160
613554
neXtProt NX_P04275
Orphanet 166078
166084
166087
166090
166093
166096
PharmGKB PA37337
eggNOG NOG12793
GeneTree ENSGT00760000118896
HOGENOM HOG000169747
HOVERGEN HBG004380
InParanoid P04275
KO K03900
OMA ECCGRCL
OrthoDB EOG73V6J9
PhylomeDB P04275
TreeFam TF300299
Reactome REACT_1230
REACT_13552
REACT_15381
REACT_15523
REACT_23847
REACT_326
ChiTaRS VWF
EvolutionaryTrace P04275
GeneWiki Von_Willebrand_factor
GenomeRNAi 7450
NextBio 29172
PRO PR:P04275
Proteomes UP000005640
Bgee P04275
CleanEx HS_VWF
ExpressionAtlas P04275
Genevestigator P04275
GO GO:0005783
GO:0009897
GO:0031012
GO:0005576
GO:0070062
GO:0031091
GO:0031093
GO:0005578
GO:0033093
GO:0051087
GO:0005518
GO:0001948
GO:0042802
GO:0019865
GO:0005178
GO:0002020
GO:0042803
GO:0047485
GO:0007596
GO:0007597
GO:0007155
GO:0031589
GO:0030198
GO:0007599
GO:0001889
GO:0001890
GO:0030168
GO:0002576
GO:0051260
GO:0009611
Gene3D 3.40.50.410
InterPro IPR006207
IPR002919
IPR014853
IPR012011
IPR002035
IPR001007
IPR001846
PANTHER PTHR11339:SF259
Pfam PF08742
PF01826
PF00092
PF00093
PF00094
SMART SM00832
SM00041
SM00327
SM00214
SM00216
SUPFAM SSF53300
SSF57567
PROSITE PS01185
PS01225
PS50234
PS01208
PS50184
PS51233

Annotations

Qualifier GO ID GO term name Reference ECO ID ECO term name with/from Aspect Extension Notes Status

enables

GO:0005201

extracellular matrix structural constituent

PMID:23979707[1]

ECO:0007666

automatically integrated combinatorial computational and experimental evidence used in manual assertion

F

occurs_in:(CL:0002543)

Seeded From UniProt

complete

part_of

GO:0062023

collagen-containing extracellular matrix

PMID:28675934[2]

ECO:0007005

high throughput direct assay evidence used in manual assertion

C

part_of:(UBERON:0003688)

Seeded From UniProt

complete

enables

GO:0005201

extracellular matrix structural constituent

PMID:28675934[2]

ECO:0007666

automatically integrated combinatorial computational and experimental evidence used in manual assertion

F

occurs_in:(UBERON:0003688)

Seeded From UniProt

complete

enables

GO:0005201

extracellular matrix structural constituent

PMID:25037231[3]

ECO:0007666

automatically integrated combinatorial computational and experimental evidence used in manual assertion

F

occurs_in:(UBERON:0001155)

Seeded From UniProt

complete

part_of

GO:0062023

collagen-containing extracellular matrix

PMID:25037231[3]

ECO:0007005

high throughput direct assay evidence used in manual assertion

C

part_of:(UBERON:0001155)

Seeded From UniProt

complete

part_of

GO:0005576

extracellular region

PMID:27068509[4]

ECO:0007005

high throughput direct assay evidence used in manual assertion

C

part_of:(UBERON:0007318)

Seeded From UniProt

complete

part_of

GO:0062023

collagen-containing extracellular matrix

PMID:23979707[1]

ECO:0007005

high throughput direct assay evidence used in manual assertion

C

part_of:(CL:0002543)

Seeded From UniProt

complete

part_of

GO:0070062

extracellular exosome

PMID:19056867[5]

ECO:0007005

high throughput direct assay evidence used in manual assertion

C

part_of:(UBERON:0001088)

Seeded From UniProt

complete

involved_in

GO:0051260

protein homooligomerization

PMID:8874190[6]

ECO:0000314

direct assay evidence used in manual assertion

P

Seeded From UniProt

complete

enables

GO:0051087

chaperone binding

PMID:10887119[7]

ECO:0000314

direct assay evidence used in manual assertion

F

Seeded From UniProt

complete

enables

GO:0047485

protein N-terminus binding

PMID:7721887[8]

ECO:0000353

physical interaction evidence used in manual assertion

UniProtKB:P07359

F

Seeded From UniProt

complete

enables

GO:0042803

protein homodimerization activity

PMID:10887119[7]

ECO:0000314

direct assay evidence used in manual assertion

F

Seeded From UniProt

complete

part_of

GO:0033093

Weibel-Palade body

PMID:3087627[9]

ECO:0000314

direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

part_of

GO:0033093

Weibel-Palade body

PMID:3082891[10]

ECO:0000314

direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

involved_in

GO:0031589

cell-substrate adhesion

PMID:9079671[11]

ECO:0000314

direct assay evidence used in manual assertion

P

Seeded From UniProt

complete

part_of

GO:0031091

platelet alpha granule

PMID:9759493[12]

ECO:0000303

author statement without traceable support used in manual assertion

C

Seeded From UniProt

complete

part_of

GO:0062023

collagen-containing extracellular matrix

PMID:6754744[13]

ECO:0000314

direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

involved_in

GO:0030168

platelet activation

PMID:8565074[14]

ECO:0000314

direct assay evidence used in manual assertion

P

Seeded From UniProt

complete

involved_in

GO:0030168

platelet activation

PMID:12871509[15]

ECO:0000303

author statement without traceable support used in manual assertion

P

Seeded From UniProt

complete

enables

GO:0019865

immunoglobulin binding

PMID:3121636[16]

ECO:0000314

direct assay evidence used in manual assertion

F

Seeded From UniProt

complete

involved_in

GO:0007599

hemostasis

PMID:10887119[7]

ECO:0000315

mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

involved_in

GO:0007596

blood coagulation

PMID:16409464[17]

ECO:0000315

mutant phenotype evidence used in manual assertion

P

Seeded From UniProt

complete

involved_in

GO:0007155

cell adhesion

PMID:10764791[18]

ECO:0000314

direct assay evidence used in manual assertion

P

Seeded From UniProt

complete

part_of

GO:0005783

endoplasmic reticulum

PMID:6754744[13]

ECO:0000314

direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

part_of

GO:0005576

extracellular region

PMID:10887119[7]

ECO:0000314

direct assay evidence used in manual assertion

C

Seeded From UniProt

complete

part_of

GO:0005576

extracellular region

PMID:14718574[19]

ECO:0000303

author statement without traceable support used in manual assertion

C

Seeded From UniProt

complete

enables

GO:0005518

collagen binding

PMID:2056120[20]

ECO:0000314

direct assay evidence used in manual assertion

F

Seeded From UniProt

complete

enables

GO:0005178

integrin binding

PMID:9079671[11]

ECO:0000353

physical interaction evidence used in manual assertion

UniProtKB:P13612

F

Seeded From UniProt

complete

enables

GO:0002020

protease binding

PMID:12775718[21]

ECO:0000353

physical interaction evidence used in manual assertion

UniProtKB:Q76LX8

F

Seeded From UniProt

complete

enables

GO:0002020

protease binding

PMID:15824096[22]

ECO:0000314

direct assay evidence used in manual assertion

F

Seeded From UniProt

complete

enables

GO:0042802

identical protein binding

PMID:21857647[23]

ECO:0000353

physical interaction evidence used in manual assertion

UniProtKB:P04275

F

Seeded From UniProt

complete

enables

GO:0042802

identical protein binding

PMID:18492805[24]

ECO:0000353

physical interaction evidence used in manual assertion

UniProtKB:P04275

F

Seeded From UniProt

complete

enables

GO:0042802

identical protein binding

PMID:18182488[25]

ECO:0000353

physical interaction evidence used in manual assertion

UniProtKB:P04275

F

Seeded From UniProt

complete

enables

GO:0042802

identical protein binding

PMID:17895385[26]

ECO:0000353

physical interaction evidence used in manual assertion

UniProtKB:P04275

F

Seeded From UniProt

complete

involved_in

GO:0009611

response to wounding

PMID:15029268[27]

ECO:0000304

author statement supported by traceable reference used in manual assertion

P

Seeded From UniProt

complete

part_of

GO:0031093

platelet alpha granule lumen

Reactome:R-HSA-481007

ECO:0000304

author statement supported by traceable reference used in manual assertion

C

Seeded From UniProt

complete

involved_in

GO:0030198

extracellular matrix organization

Reactome:R-HSA-1474244

ECO:0000304

author statement supported by traceable reference used in manual assertion

P

Seeded From UniProt

complete

involved_in

GO:0030168

platelet activation

Reactome:R-HSA-76002

ECO:0000304

author statement supported by traceable reference used in manual assertion

P

Seeded From UniProt

complete

involved_in

GO:0007597

blood coagulation, intrinsic pathway

Reactome:R-HSA-140837

ECO:0000304

author statement supported by traceable reference used in manual assertion

P

Seeded From UniProt

complete

involved_in

GO:0007596

blood coagulation

Reactome:R-HSA-109582

ECO:0000304

author statement supported by traceable reference used in manual assertion

P

Seeded From UniProt

complete

part_of

GO:0005576

extracellular region

Reactome:R-HSA-481007
Reactome:R-HSA-443418
Reactome:R-HSA-443402
Reactome:R-HSA-430096
Reactome:R-HSA-430076
Reactome:R-HSA-430073
Reactome:R-HSA-429529
Reactome:R-HSA-429441
Reactome:R-HSA-429415
Reactome:R-HSA-377644
Reactome:R-HSA-377643
Reactome:R-HSA-377641
Reactome:R-HSA-377640
Reactome:R-HSA-372705
Reactome:R-HSA-372697
Reactome:R-HSA-372693
Reactome:R-HSA-354165
Reactome:R-HSA-354149
Reactome:R-HSA-354124
Reactome:R-HSA-354087
Reactome:R-HSA-354073
Reactome:R-HSA-354066
Reactome:R-HSA-265425
Reactome:R-HSA-216072
Reactome:R-HSA-158137
Reactome:R-HSA-158118
Reactome:R-HSA-114671
Reactome:R-HSA-114670

ECO:0000304

author statement supported by traceable reference used in manual assertion




























C

Seeded From UniProt

complete

involved_in

GO:0002576

platelet degranulation

Reactome:R-HSA-114608

ECO:0000304

author statement supported by traceable reference used in manual assertion

P

Seeded From UniProt

complete

involved_in

GO:0007599

hemostasis

GO_REF:0000037

ECO:0000322

imported manually asserted information used in automatic assertion

UniProtKB-KW:KW-0356

P

Seeded From UniProt

complete

part_of

GO:0005576

extracellular region

GO_REF:0000037
GO_REF:0000039

ECO:0000322

imported manually asserted information used in automatic assertion

UniProtKB-KW:KW-0964
UniProtKB-SubCell:SL-0243

C

Seeded From UniProt

complete

involved_in

GO:0007596

blood coagulation

GO_REF:0000037

ECO:0000322

imported manually asserted information used in automatic assertion

UniProtKB-KW:KW-0094

P

Seeded From UniProt

complete

involved_in

GO:0007155

cell adhesion

GO_REF:0000037

ECO:0000322

imported manually asserted information used in automatic assertion

UniProtKB-KW:KW-0130

P

Seeded From UniProt

complete

edit table

Notes

References

See Help:References for how to manage references in GONUTS.

  1. ↑ 1.0 1.1 Zanivan, S et al. (2013) SILAC-based proteomics of human primary endothelial cell morphogenesis unveils tumor angiogenic markers. Mol. Cell Proteomics 12 3599-611 PubMed GONUTS page
  2. ↑ 2.0 2.1 Naba, A et al. (2017) Characterization of the Extracellular Matrix of Normal and Diseased Tissues Using Proteomics. J. Proteome Res. 16 3083-3091 PubMed GONUTS page
  3. ↑ 3.0 3.1 Naba, A et al. (2014) Extracellular matrix signatures of human primary metastatic colon cancers and their metastases to liver. BMC Cancer 14 518 PubMed GONUTS page
  4. ↑ Barallobre-Barreiro, J et al. (2016) Extracellular matrix remodelling in response to venous hypertension: proteomics of human varicose veins. Cardiovasc. Res. 110 419-30 PubMed GONUTS page
  5. ↑ Gonzales, PA et al. (2009) Large-scale proteomics and phosphoproteomics of urinary exosomes. J. Am. Soc. Nephrol. 20 363-79 PubMed GONUTS page
  6. ↑ Siedlecki, CA et al. (1996) Shear-dependent changes in the three-dimensional structure of human von Willebrand factor. Blood 88 2939-50 PubMed GONUTS page
  7. ↑ 7.0 7.1 7.2 7.3 Allen, S et al. (2000) A novel von Willebrand disease-causing mutation (Arg273Trp) in the von Willebrand factor propeptide that results in defective multimerization and secretion. Blood 96 560-8 PubMed GONUTS page
  8. ↑ Marchese, P et al. (1995) Identification of three tyrosine residues of glycoprotein Ib alpha with distinct roles in von Willebrand factor and alpha-thrombin binding. J. Biol. Chem. 270 9571-8 PubMed GONUTS page
  9. ↑ Sporn, LA et al. (1986) Inducible secretion of large, biologically potent von Willebrand factor multimers. Cell 46 185-90 PubMed GONUTS page
  10. ↑ Wagner, DD et al. (1986) Initial glycosylation and acidic pH in the Golgi apparatus are required for multimerization of von Willebrand factor. J. Cell Biol. 102 1320-4 PubMed GONUTS page
  11. ↑ 11.0 11.1 Isobe, T et al. (1997) Propolypeptide of von Willebrand factor is a novel ligand for very late antigen-4 integrin. J. Biol. Chem. 272 8447-53 PubMed GONUTS page
  12. ↑ Sadler, JE (1998) Biochemistry and genetics of von Willebrand factor. Annu. Rev. Biochem. 67 395-424 PubMed GONUTS page
  13. ↑ 13.0 13.1 Wagner, DD et al. (1982) Immunolocalization of von Willebrand protein in Weibel-Palade bodies of human endothelial cells. J. Cell Biol. 95 355-60 PubMed GONUTS page
  14. ↑ Savage, B et al. (1996) Initiation of platelet adhesion by arrest onto fibrinogen or translocation on von Willebrand factor. Cell 84 289-97 PubMed GONUTS page
  15. ↑ Song, S et al. (2003) von Willebrand factor (VWF)-dependent human platelet activation: porcine VWF utilizes different transmembrane signaling pathways than does thrombin to activate platelets, but both require protein phosphatase function. J. Thromb. Haemost. 1 337-46 PubMed GONUTS page
  16. ↑ Dorner, AJ et al. (1987) The relationship of N-linked glycosylation and heavy chain-binding protein association with the secretion of glycoproteins. J. Cell Biol. 105 2665-74 PubMed GONUTS page
  17. ↑ Hommais, A et al. (2006) Mutations C1157F and C1234W of von Willebrand factor cause intracellular retention with defective multimerization and secretion. J. Thromb. Haemost. 4 148-57 PubMed GONUTS page
  18. ↑ Cruz, MA et al. (2000) Mapping the glycoprotein Ib-binding site in the von willebrand factor A1 domain. J. Biol. Chem. 275 19098-105 PubMed GONUTS page
  19. ↑ Anderson, NL et al. (2004) The human plasma proteome: a nonredundant list developed by combination of four separate sources. Mol. Cell Proteomics 3 311-26 PubMed GONUTS page
  20. ↑ Rand, JH et al. (1991) 150-kD von Willebrand factor binding protein extracted from human vascular subendothelium is type VI collagen. J. Clin. Invest. 88 253-9 PubMed GONUTS page
  21. ↑ Dong, JF et al. (2003) ADAMTS-13 metalloprotease interacts with the endothelial cell-derived ultra-large von Willebrand factor. J. Biol. Chem. 278 29633-9 PubMed GONUTS page
  22. ↑ Majerus, EM et al. (2005) Binding of ADAMTS13 to von Willebrand factor. J. Biol. Chem. 280 21773-8 PubMed GONUTS page
  23. ↑ Zhou, YF et al. (2011) A pH-regulated dimeric bouquet in the structure of von Willebrand factor. EMBO J. 30 4098-111 PubMed GONUTS page
  24. ↑ Cao, W et al. (2008) Factor VIII accelerates proteolytic cleavage of von Willebrand factor by ADAMTS13. Proc. Natl. Acad. Sci. U.S.A. 105 7416-21 PubMed GONUTS page
  25. ↑ Huang, RH et al. (2008) Assembly of Weibel-Palade body-like tubules from N-terminal domains of von Willebrand factor. Proc. Natl. Acad. Sci. U.S.A. 105 482-7 PubMed GONUTS page
  26. ↑ Purvis, AR et al. (2007) Two Cys residues essential for von Willebrand factor multimer assembly in the Golgi. Proc. Natl. Acad. Sci. U.S.A. 104 15647-52 PubMed GONUTS page
  27. ↑ Ruggeri, ZM & Ruggeri, ZM (2004) Platelet and von Willebrand factor interactions at the vessel wall. Hamostaseologie 24 1-11 PubMed GONUTS page
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