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  von Willebrand factor

SourceCanis lupus familiaris (dog)
Taxonomy Canis lupus familiaris Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Laurasiatheria; Carnivora; Caniformia; Canidae; Canis.
KeywordsBlood coagulation; Cell adhesion; Cleavage on pair of basic residues; Disulfide bond; Extracellular matrix; Glycoprotein; Hemostasis; Repeat; Secreted; Signal.
Details
Function: Important in the maintenance of hemostasis, it promotes adhesion of platelets to the sites of vascular injury by forming a molecular bridge between sub-endothelial collagen matrix and platelet-surface receptor complex, glycoprotein Ibalpha/IX/V. Also acts as a chaperone for coagulation factor VIII, delivering it to the site of injury, stabilizing its heterodimeric structure and protecting it from premature clearance from plasma (By similarity).

Post-translational modification: All cysteine residues are involved in intrachain or interchain disulfide bonds (By similarity). N- and O-glycosylated (By similarity).

Similarity: Contains 1 CTCK (C-terminal cystine knot-like) domain. Contains 4 TIL (trypsin inhibitory-like) domains. Contains 3 VWFA domains. Contains 3 VWFC domains. Contains 4 VWFD domains.

Subcellular location: Secreted. Secreted, extracellular space, extracellular matrix. Note=Localized to storage granules.

Subunit structure: Multimeric. Interacts with F8.

Tissue specificity: Plasma.

Disease: Defects in VWF are the cause of von Willebrand disease (VWD) in the Scottish Terrier. VWD is characterized by frequent bleeding. Type I VWD is associated with a deficiency of VWF; type II by normal to decreased plasma level of VWF; type III by a virtual absence of VWF.

Sequence length: 2813 AA.

Sequence
MSPTRLVRVLLALALILPGKLCTKGTVGRSSMARCSLFGGDFINTFDESMYSFAGDCSYL
LAGDCQEHSVSLIGGFQNGKRVSLSVYLGEFFDIHLFVNGTMLQGTQSISMPYASNGLYL
EAEAGYYKLSSEAYGFVARIDGNGNFQVLLSDRYFNKTCGLCGNFNIFAEDDFRTQEGTL
TSDPYDFANSWALSSGEQRCKRVSPPSSPCNVSSDEVQQVLWEQCQLLKSASVFARCHPL
VDPEPFVALCERTLCTCVQGMECPCAVLLEYARACAQQGIVLYGWTDHSVCRPACPAGME
YKECVSPCTRTCQSLHVKEVCQEQCVDGCSCPEGQLLDEGHCVGSAECSCVHAGQRYPPG
ASLLQDCHTCICRNSLWICSNEECPGECLVTGQSHFKSFDNRYFTFSGVCHYLLAQDCQD
HTFSVVIETVQCADDLDAVCTRSVTVRLPGHHNSLVKLKHGGGVSMDGQDIQIPLLQGDL
RIQHTVMASVRLSYGEDLQMDWDGRGRLLVTLSPAYAGKTCGLCGNYNGNRGDDFVTPAG
LAEPLVEDFGNAWKLLGACENLQKQHRDPCSLNPRQARFAEEACALLTSSKFEPCHRAVG
PQPYVQNCRYDVCSCSDGRDCLCSAVANYAAACARRGVHIAWREPGFCALSCPQGQVYLQ
CGTPCNMTCRSLSYPEEDCNEVCLEGCFCPPGLYLDERGDCVPKAQCPCYYDGEIFQPED
IFSDHHTMCYCEDGFMHCTTSGGLGSLLPNPVLSSPRSHRSKRSLSCRPPMVKLVCPADN
PRAEGLECAKTCQNYDLQCMSTGCVSGCLCPQGMVRHENRCVALERCPCFHQGQEYAPGE
TVKIDCNTCVCRDRKWNCTDHVCDATCSAIGMAHYLTFDGLKYLFPGECQYVLVQDYCGS
NPGTFRILVGNEGCSYPSVKCKKRVTILVEGGEIELFDGEVNVKKPMKDETHFEVVESGQ
YVILLLGKALSVVWDHRLSISVTLKRTYQEQVCGLCGNFDGIQNNDFTSSSLQIEEDPVD
FGNSWKVNPQCADTKKVPLDSSPAVCHNNIMKQTMVDSSCRILTSDIFQDCNRLVDPEPF
LDICIYDTCSCESIGDCTCFCDTIAAYAHVCAQHGKVVAWRTATFCPQNCEERNLHENGY
ECEWRYNSCAPACPITCQHPEPLACPVQCVEGCHAHCPPGKILDELLQTCIDPEDCPVCE
VAGRRLAPGKKIILNPSDPEHCQICHCDGVNFTCQACREPGSLVVPPTEGPIGSTTSYVE
DTPEPPLHDFHCSRLLDLVFLLDGSSKLSEDEFEVLKVFVVGMMEHLHISQKRIRVAVVE
YHDGSHAYIELKDRKRPSELRRITSQVKYAGSEVASTSEVLKYTLFQIFGKIDRPEASRI
ALLLMASQEPSRLARNLVRYVQGLKKKKVIVIPVGIGPHASLKQIHLIEKQAPENKAFVF
SGVDELEQRRDEIINYLCDLAPEAPAPTQHPPMAQVTVGSELLGVSSPGPKRNSMVLDVV
FVLEGSDKIGEANFNKSREFMEEVIQRMDVGQDRIHVTVLQYSYMVTVEYTFSEAQSKGE
VLQQVRDIRYRGGNRTNTGLALQYLSEHSFSVSQGDREQVPNLVYMVTGNPASDEIKRMP
GDIQVVPIGVGPHANVQELEKIGWPNAPILIHDFEMLPREAPDLVLQRCCSGEGLQIPTL
SPTPDCSQPLDVVLLLDGSSSIPASYFDEMKSFTKAFISRANIGPRLTQVSVLQYGSITT
IDVPWNVAYEKVHLLSLVDLMQQEGGPSQIGDALSFAVRYVTSEVHGARPGASKAVVILV
TDVSVDSVDAAAEAARSNRVTVFPIGIGDRYSEAQLSSLAGPKAGSNMVRLQRIEDLPTV
ATLGNSFFHKLCSGFDRVCVDEDGNEKRPGDVWTLPDQCHTVTCLPDGQTLLKSHRVNCD
RGPRPSCPNGQPPLRVEETCGCRWTCPCVCMGSSTRHIVTFDGQNFKLTGSCSYVLFQNK
EQDLEVILHNGACSPGAKETCMKSIEVKHDGLSVELHSDMQMTVNGRLVSIPYVGGDMEV
NVYGTIMYEVRFNHLGHIFTFTPQNNEFQLQLSPRTFASKTYGLCGICDENGANDFILRD
GTVTTDWKALIQEWTVQQLGKTCQPVPEEQCPVSSSSHCQVLLSELFAECHKVLAPATFY
AMCQPDSCHPKKVCEAIALYAHLCRTKGVCVDWRRANFCAMSCPPSLVYNHCEHGCPRLC
EGNTSSCGDQPSEGCFCPPNQVMLEGSCVPEEACTQCISEDGVRHQFLETWVPAHQPCQI
CTCLSGRKVNCTLQPCPTARAPTCGPCEVARLRQNAEQCCPEYECVCDLVSCDLPPVPPC
EDGLQMTLTNPGECRPNFTCACRKDECRRESPPSCPPHRTLALRKTQCCDEYECACNCVN
STVSCPLGYLASAVTNDCGCTTTTCFPDKVCVHRGTIYPVGQFWEEACDVCTCTDLEDSV
MGLRVAQCSQKPCEDNCLSGFTYVLHEGECCGRCLPSACEVVIGSPRGDAQSHWKNVGSH
WASPDNPCLINECVRVKEEVFVQQRNVSCPQLNVPTCPTGFQLSCKTSECCPTCHCEPLE
ACLLNGTIIGPGKSLMIDVCTTCRCTVQVGVISGFKLECRKTTCEACPLGYKEEKNQGEC
CGRCLPIACTIQLRGGQIMTLKRDETIQDGCDSHFCKVNERGEYIWEKRVTGCPPFDEHK
CLAEGGKIMKIPGTCCDTCEEPECKDIIAKLQRVKVGDCKSEEEVDIHYCEGKCASKAVY
SIHMEDVQDQCSCCSPTQTEPMQVPLRCTNGSLIYHEILNAMQCRCSPRKCSK
Accession NumberQ28295 
PubMed ID10961880, 10668811, 12871266 
CTD DB399544
eggNOG DBmaNOG13467
Ensembl DBENSCAFT00000024188
GeneID DB399544
GO DB0005783, 0005578, 0033093, 0051087, 0005518, 0001948, 0019865, 0005178, 0002020, 0042803, 0047485, 0031589, 0030168, 0051260
InterPro DBIPR014853, IPR006207, IPR002919, IPR018453, IPR001846, IPR012011, IPR002035, IPR001007
KEGGcfa:399544
NCBIL76227, AAB05549, U66246.2, AAB93766.2, AF099154, AAD04919, L16903, AAA30903
OMIM105200, 134820, 202400, 134830, 202400, 134850, 202400, 176930, 601367, 134390, 188055, 227400, 600880, 601367, 612309, 227500, 134500, 306700, 300746, 306900, 227600, 176860, 188050, 612283, 612304, 176880, 612336, 264900, 612416, 234000, 610618, 610619, 229000, 612423, 107300, 188050, 134570, 134580, 193400, 277480
PfamPF08742, PF01826, PF00092, PF00093, PF00094
PROSITE DBPS01185, PS01225, PS50234, PS01208, PS50184, PS51233
SMART DBSM00832, SM00041, SM00327, SM00214, SM00216
UniGeneCfa.111



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