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VWF(764-2813) [extracellular region]
Stable Identifier
R-HSA-158327
Type
Protein [EntityWithAccessionedSequence]
Species
Homo sapiens
Compartment
extracellular region
Synonyms
von Willebrand factor, vWF
Icon
Locations in the PathwayBrowser
Expand all
Disease (Homo sapiens)
Diseases of hemostasis (Homo sapiens)
Defects of contact activation system (CAS) and kallikrein/kinin system (KKS) (Homo sapiens)
Defective factor VIII causes hemophilia A (Homo sapiens)
Defective F8 binding to von Willebrand factor (Homo sapiens)
F8 variant does not bind von Willebrand factor (Homo sapiens)
VWF multimer [extracellular region] (Homo sapiens)
VWF(764-2813) [extracellular region] (Homo sapiens)
Defective F8 cleavage by thrombin (Homo sapiens)
F8 variant is not cleaved by thrombin (Homo sapiens)
FVIII variant:vWF multimer [extracellular region] (Homo sapiens)
VWF(764-2813) [extracellular region] (Homo sapiens)
Defects of platelet adhesion to exposed collagen (Homo sapiens)
Defective VWF cleavage by ADAMTS13 variant (Homo sapiens)
ADAMTS13 variant does not cleave VWF multimer (Homo sapiens)
Collagen type I fibril:VWF multimer [extracellular region] (Homo sapiens)
VWF multimer [extracellular region] (Homo sapiens)
VWF(764-2813) [extracellular region] (Homo sapiens)
Enhanced binding of GP1BA variant to VWF multimer:collagen (Homo sapiens)
GP1BA variant binds to VWF multimer:collagen (Homo sapiens)
Caplacizumab:VWF:collagen type I [extracellular region] (Homo sapiens)
Collagen type I fibril:VWF multimer [extracellular region] (Homo sapiens)
VWF multimer [extracellular region] (Homo sapiens)
VWF(764-2813) [extracellular region] (Homo sapiens)
Collagen type I fibril:VWF multimer [extracellular region] (Homo sapiens)
VWF multimer [extracellular region] (Homo sapiens)
VWF(764-2813) [extracellular region] (Homo sapiens)
Collagen type I fibril:VWF multimer:GP1BA variant:GP1BB:GP9:GP5 [plasma membrane] (Homo sapiens)
Collagen type I fibril:VWF multimer [extracellular region] (Homo sapiens)
VWF multimer [extracellular region] (Homo sapiens)
VWF(764-2813) [extracellular region] (Homo sapiens)
Hemostasis (Homo sapiens)
Formation of Fibrin Clot (Clotting Cascade) (Homo sapiens)
Intrinsic Pathway of Fibrin Clot Formation (Homo sapiens)
factor VIII + von Willebrand factor multimer -> factor VIII:von Willibrand factor multimer (Homo sapiens)
VWF multimer [extracellular region] (Homo sapiens)
VWF(764-2813) [extracellular region] (Homo sapiens)
factor VIII:von Willebrand factor multimer [extracellular region] (Homo sapiens)
VWF(764-2813) [extracellular region] (Homo sapiens)
factor VIII:von Willibrand factor multimer -> factor VIIIa + factor VIIIa B A3 acidic polypeptide + von Willibrand factor multimer (Homo sapiens)
VWF multimer [extracellular region] (Homo sapiens)
VWF(764-2813) [extracellular region] (Homo sapiens)
factor VIII:von Willebrand factor multimer [extracellular region] (Homo sapiens)
VWF(764-2813) [extracellular region] (Homo sapiens)
Platelet Adhesion to exposed collagen (Homo sapiens)
ADAMTS13 cleaves VWF multimer (Homo sapiens)
Collagen type I fibril:VWF multimer [extracellular region] (Homo sapiens)
VWF multimer [extracellular region] (Homo sapiens)
VWF(764-2813) [extracellular region] (Homo sapiens)
Collagen type I fibril:cleaved VWF multimer [extracellular region] (Homo sapiens)
cleaved VWF multimer [extracellular region] (Homo sapiens)
VWF multimer:VWF(1606-2813) [extracellular region] (Homo sapiens)
VWF(764-2813) [extracellular region] (Homo sapiens)
VWF multimer:VWF(764-1605) [extracellular region] (Homo sapiens)
VWF(764-2813) [extracellular region] (Homo sapiens)
Caplacizumab binds VWF (Homo sapiens)
Caplacizumab:VWF:collagen type I [extracellular region] (Homo sapiens)
Collagen type I fibril:VWF multimer [extracellular region] (Homo sapiens)
VWF multimer [extracellular region] (Homo sapiens)
VWF(764-2813) [extracellular region] (Homo sapiens)
Collagen type I fibril:VWF multimer [extracellular region] (Homo sapiens)
VWF multimer [extracellular region] (Homo sapiens)
VWF(764-2813) [extracellular region] (Homo sapiens)
GPIb:IX:V binds to VWF multimer:collagen (Homo sapiens)
Caplacizumab:VWF:collagen type I [extracellular region] (Homo sapiens)
Collagen type I fibril:VWF multimer [extracellular region] (Homo sapiens)
VWF multimer [extracellular region] (Homo sapiens)
VWF(764-2813) [extracellular region] (Homo sapiens)
Collagen type I fibril:VWF multimer [extracellular region] (Homo sapiens)
VWF multimer [extracellular region] (Homo sapiens)
VWF(764-2813) [extracellular region] (Homo sapiens)
Collagen type I fibril:cleaved VWF multimer [extracellular region] (Homo sapiens)
cleaved VWF multimer [extracellular region] (Homo sapiens)
VWF multimer:VWF(1606-2813) [extracellular region] (Homo sapiens)
VWF(764-2813) [extracellular region] (Homo sapiens)
VWF multimer:VWF(764-1605) [extracellular region] (Homo sapiens)
VWF(764-2813) [extracellular region] (Homo sapiens)
GP1BA:GP1BB:GP9:GP5:VWF:collagen type I [plasma membrane] (Homo sapiens)
Collagen type I fibril:VWF multimer [extracellular region] (Homo sapiens)
VWF multimer [extracellular region] (Homo sapiens)
VWF(764-2813) [extracellular region] (Homo sapiens)
cleaved VWF multimer [extracellular region] (Homo sapiens)
VWF multimer:VWF(1606-2813) [extracellular region] (Homo sapiens)
VWF(764-2813) [extracellular region] (Homo sapiens)
VWF multimer:VWF(764-1605) [extracellular region] (Homo sapiens)
VWF(764-2813) [extracellular region] (Homo sapiens)
VWF multimer binds to collagen type I (Homo sapiens)
Collagen type I fibril:VWF multimer [extracellular region] (Homo sapiens)
VWF multimer [extracellular region] (Homo sapiens)
VWF(764-2813) [extracellular region] (Homo sapiens)
VWF multimer [extracellular region] (Homo sapiens)
VWF(764-2813) [extracellular region] (Homo sapiens)
External Reference Information
External Reference
UniProt:P04275 VWF
Gene Names
VWF, F8VWF
Chain
signal peptide:1-22, chain:23-763, chain:764-2813
Reference Genes
BioGPS Gene:7450 P042752
COSMIC (genes):VWF VWF
CTD Gene:7450 P042752
dbSNP Gene:7450 VWF
ENSEMBL:ENSG00000110799 VWF
HGNC:12726 P042752
KEGG:hsa:7450 P042752
Monarch:7450 P042752
NCBI Gene:7450 P042752
OMIM:613160 P042752
UCSC:P04275 P042752
Reference Transcript
RefSeq:XM_047429501.1 P042752
RefSeq:NM_000552.5 P042752
Participates
as a component of
FVIII variant:vWF multimer [extracellular region] (Homo sapiens)
VWF multimer:VWF(1606-2813) [extracellular region] (Homo sapiens)
VWF multimer:VWF(764-1605) [extracellular region] (Homo sapiens)
VWF multimer [extracellular region] (Homo sapiens)
factor VIII:von Willebrand factor multimer [extracellular region] (Homo sapiens)
as a repeated unit of
VWF multimer [extracellular region] (Homo sapiens)
Other forms of this molecule
VWF(23-763) [extracellular region]
VWF(23-763) [platelet alpha granule lumen]
VWF L1733P (764-2813) [extracellular region]
VWF S1731L (764-2813) [extracellular region]
VWF K1794E (764-2813) [extracellular region]
VWF S1731T (764-2813) [extracellular region]
VWF S1783A (764-2813) [extracellular region]
VWF H1786D (764-2813) [extracellular region]
VWF W1745C (764-2813) [extracellular region]
VWF(764-1605) [extracellular region]
VWF (1606-2813) [extracellular region]
VWF G1579R (764-2813) [extracellular region]
VWF Y1584C (764-2813) [extracellular region]
VWF G1631D (764-2813) [extracellular region]
VWF C1099P (764-2813) [extracellular region]
VWF I1568N (764-2813) [extracellular region]
VWF Y1584C (764-1605) [extracellular region]
VWF G1631D (1606-2813) [extracellular region]
VWF I1568N (764-1605) [extracellular region]
VWF C1099P (764-1605) [extracellular region]
VWF G1579R (764-1605) [extracellular region]
VWF S1358N (764-2813) [extracellular region]
VWF S1387I (764-2813) [extracellular region]
VWF S1394F (764-2813) [extracellular region]
VWF Q1402P (764-2813) [extracellular region]
Inferred To
VWF [extracellular region]
(Bos taurus)
VWF [extracellular region]
(Canis familiaris)
VWF [extracellular region]
(Gallus gallus)
VWF [extracellular region]
(Sus scrofa)
vwf [extracellular region]
(Danio rerio)
vwf [extracellular region]
(Xenopus tropicalis)
Vwf [extracellular region]
(Mus musculus)
Cross References
RefSeq
NP_000543.3
,
XP_047285457.1
OpenTargets
ENSG00000110799
ENSEMBL
ENST00000261405
,
ENSG00000110799
,
ENSP00000261405
GeneCards
VWF
HPA
ENSG00000110799-VWF
ZINC - Substances
VWF_HUMAN
ZINC target
P04275
PRO
P04275
Pharos - Targets
P04275
GlyGen
P04275
Orphanet
VWF
PDB
1IJB
,
7PNF
,
1ATZ
,
4DMU
,
3HXO
,
1UEX
,
1AO3
,
6FWN
,
1SQ0
,
7PMV
,
1OAK
,
3ZQK
,
2MHQ
,
2MHP
,
1FE8
,
3PPY
,
3PPX
,
7P4N
,
5BV8
,
8D3D
,
1AUQ
,
8D3C
,
4C2A
,
1FNS
,
3HXQ
,
3PPW
,
1M10
,
4NT5
,
3PPV
,
1IJK
,
7F49
,
2ADF
,
7KWO
,
6N29
,
4C2B
,
4C29
,
7EOW
,
3GXB
,
7ZWH
,
1U0N
Interactors (6)
Accession
#Entities
Entities
Confidence Score
Evidence (IntAct)
UniProt:P04275 VWF
20
VWF(23-763) [extracellular region]
(R-HSA-67271)
VWF(764-2813) [extracellular region]
(R-HSA-158327)
VWF(23-763) [platelet alpha granule lumen]
(R-HSA-114662)
VWF L1733P (764-2813) [extracellular region]
(R-HSA-9824345)
VWF S1731L (764-2813) [extracellular region]
(R-HSA-9823681)
VWF K1794E (764-2813) [extracellular region]
(R-HSA-9823657)
VWF S1731T (764-2813) [extracellular region]
(R-HSA-9823615)
VWF S1783A (764-2813) [extracellular region]
(R-HSA-9823588)
VWF H1786D (764-2813) [extracellular region]
(R-HSA-9823634)
VWF W1745C (764-2813) [extracellular region]
(R-HSA-9823611)
VWF(764-1605) [extracellular region]
(R-HSA-9822545)
VWF (1606-2813) [extracellular region]
(R-HSA-9822534)
VWF G1579R (764-2813) [extracellular region]
(R-HSA-9844214)
VWF Y1584C (764-2813) [extracellular region]
(R-HSA-9844202)
VWF G1631D (764-2813) [extracellular region]
(R-HSA-9844230)
VWF C1099P (764-2813) [extracellular region]
(R-HSA-9844221)
VWF I1568N (764-2813) [extracellular region]
(R-HSA-9844203)
VWF Y1584C (764-1605) [extracellular region]
(R-HSA-9852765)
VWF G1631D (1606-2813) [extracellular region]
(R-HSA-9852788)
VWF I1568N (764-1605) [extracellular region]
(R-HSA-9852790)
0.868
21
UniProt:Q76LX8 ADAMTS13
7
glc-fuc-ADAMTS13 [extracellular region]
(R-HSA-9822541)
ADAMTS13 A250V [extracellular region]
(R-HSA-9824393)
ADAMTS13 Q449* [extracellular region]
(R-HSA-9824391)
ADAMTS13 P475S [extracellular region]
(R-HSA-9824387)
O-fuc-ADAMTS13 [endoplasmic reticulum lumen]
(R-HSA-5173286)
glc-fuc-ADAMTS13 [endoplasmic reticulum lumen]
(R-HSA-5172961)
ADAMTS13 [endoplasmic reticulum lumen]
(R-HSA-5173292)
0.789
19
UniProt:P00451 F8
20
F8(392-759) D579A [plasma membrane]
(R-HSA-9668364)
F8(392-759) V578A [plasma membrane]
(R-HSA-9668367)
F8(392-759) R546W [plasma membrane]
(R-HSA-9668360)
F8(392-759) S577F [plasma membrane]
(R-HSA-9668362)
F8(392-759) I585T [plasma membrane]
(R-HSA-9668357)
F8 N637S [endoplasmic reticulum lumen]
(R-HSA-9661996)
F8 G2013R [endoplasmic reticulum lumen]
(R-HSA-9666350)
F8 W604R [endoplasmic reticulum lumen]
(R-HSA-9661993)
F8 R1126W [endoplasmic reticulum lumen]
(R-HSA-9662699)
F8 R2326L [endoplasmic reticulum lumen]
(R-HSA-9661995)
F8 P947R [endoplasmic reticulum lumen]
(R-HSA-9662692)
F8 H1066Y [endoplasmic reticulum lumen]
(R-HSA-9662693)
F8 S603G [endoplasmic reticulum lumen]
(R-HSA-9661989)
F8 E1057K [endoplasmic reticulum lumen]
(R-HSA-9662694)
F8 E600D [endoplasmic reticulum lumen]
(R-HSA-9661991)
F8 W604C [endoplasmic reticulum lumen]
(R-HSA-9661984)
F8 D845E [endoplasmic reticulum lumen]
(R-HSA-9662689)
F8 G720S [endoplasmic reticulum lumen]
(R-HSA-9661985)
F8 E600Q [endoplasmic reticulum lumen]
(R-HSA-9662015)
F8 R2326Q [endoplasmic reticulum lumen]
(R-HSA-9661945)
0.623
2
UniProt:P07359 GP1BA
6
GP1BA [plasma membrane]
(R-HSA-55861)
GP1BA D251Y [plasma membrane]
(R-HSA-9844197)
GP1BA W246L [plasma membrane]
(R-HSA-9844258)
GP1BA M255V [plasma membrane]
(R-HSA-9844240)
GP1BA G249V [plasma membrane]
(R-HSA-9844257)
GP1BA G249S [plasma membrane]
(R-HSA-9844280)
0.623
2
UniProt:P00451-PRO_0000002967 F8
0.544
2
UniProt:Q96CV9 OPTN
6
p-S177,S473,S513-OPTN [cytosol]
(R-HSA-9687840)
OPTN [cytosol]
(R-HSA-9687907)
OPTN [Golgi membrane]
(R-HSA-2161111)
p-S177-OPTN [cytosol]
(R-HSA-2562525)
p-S177-OPTN [nucleoplasm]
(R-HSA-2562595)
OPTN [recycling endosome membrane]
(R-HSA-8854141)
0.454
2
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