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  von Willebrand factor

SourceOryctolagus cuniculus (rabbit)
Taxonomy Oryctolagus cuniculus Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Lagomorpha; Leporidae; Oryctolagus.
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 1 pacifastin repeat. Contains 3 TIL (trypsin inhibitory-like) domains. Contains 3 VWFA domains. Contains 3 VWFC domains. Contains 3 VWFD domains.

Subcellular location: Secreted (By similarity). Secreted, extracellular space, extracellular matrix (By similarity). Note=Localized to storage granules (By similarity).

Subunit structure: Multimeric. Interacts with F8 (By similarity).

Tissue specificity: Plasma.

Sequence length: 366 AA.

Sequence
QEPGGMVVPPTDAPVRSTTPYMEDTPEPPLHDFYWSNLMDLVFLLDGSAQLSEAEFGVLK
AFVVSVMERLHISQKRIRVAVVEYHDGSHSYISLKDRKRPSELRRIASQVKYAGGPVAST
SEVLKYTLFHIFSNVDRPEASRIALLLSASQETPRMVRNLVRYAQGLKKEKVIVIPVGIG
PHVSLRQIHLIEKQAPENKAFVLSGVDELEQRRDEIISYLCDLGPEAPVPTQRPPTARVT
VSPGQLGVSPQGPKRCSMVLDVVFVLEGSDEVGEANFNKSKAFVEEVIRRMDVGPGSIHV
TVLQYSYVVTVEYTFSEPQSKDDILRHVREIRYQGGNRTNTGLALQYLSEHSFTTSQGDR
EQAPNL
Accession NumberQ28639 
PubMed ID8673300 
InterPro DBIPR002035
NCBIU31618, AAB51555
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
PfamPF00092
PROSITE DBPS50234
SMART DBSM00327



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