The von Willebrand factor is a large multimeric glycoprotein

Glycoprotein

Glycoproteins are proteins that contain oligosaccharide chains covalently attached to polypeptide side-chains. The carbohydrate is attached to the protein in a cotranslational or posttranslational modification. This process is known as glycosylation. In proteins that have segments extending...

 found in blood plasma. Mutant forms are involved in the aetiology of bleeding disorders. In von Willebrand factor, the type A domain (vWF) is the prototype for a protein superfamily. The vWF domain is found in various plasma proteins: complement factors B, C2, CR3 and CR4; the integrins (I-domains); collagen types VI, VII, XII and XIV; and other extracellular proteins. Although the majority of VWA-containing proteins are extracellular, the most ancient ones present in all eukaryotes are all intracellular proteins involved in functions such as transcription, DNA repair, ribosomal and membrane transport and the proteasome

Proteasome

Proteasomes are very large protein complexes inside all eukaryotes and archaea, and in some bacteria.  In eukaryotes, they are located in the nucleus and the cytoplasm.  The main function of the proteasome is to degrade unneeded or damaged proteins by proteolysis, a chemical reaction that breaks...

. A common feature appears to be involvement in multiprotein complexes. Proteins that incorporate vWF domains participate in numerous biological events (e.g. cell adhesion, migration, homing, pattern formation, and signal transduction), involving interaction with a large array of ligands. A number of human diseases arise from mutations in VWA domains. Secondary structure prediction from 75 aligned vWF sequences has revealed a largely alternating sequence of alpha-helices and beta-strands. Fold recognition algorithms were used to score sequence compatibility with a library of known structures: the vWF domain fold was predicted to be a doubly wound, open, twisted beta-sheet flanked by alpha-helices. 3D structures have been determined for the I-domains of integrins CD11b (with bound magnesium) and CD11a (with bound manganese). The domain adopts a classic alpha/beta Rossmann fold and contains an unusual metal ion coordination site at its surface. It has been suggested that this site represents a general metal ion-dependent adhesion site (MIDAS) for binding protein ligands. The residues constituting the MIDAS motif in the CD11b and CD11a I-domains are completely conserved, but the manner in which the metal ion is coordinated differs slightly.

Human proteins containing this domain

ANTXR1

ANTXR1

Anthrax toxin receptor 1 is a protein that in humans is encoded by the ANTXR1 gene.-Further reading:...

; ANTXR2

ANTXR2

Anthrax toxin receptor 2 is a protein that in humans is encoded by the ANTXR2 gene.-Further reading:...

; BF

BF (protein)

Complement factor B is a protein that in humans is encoded by the CFB gene.-Further reading:-External links:* * * The MEROPS online database for peptidases and their inhibitors:...

; C2

C2

-Biology:* C2 domain, a protein structural domain* C2 regulatory sequence for the insulin gene* Apolipoprotein C2, a human apolipoprotein* In human anatomy, C2 may refer to:** Cervical vertebra 2, the axis, one of the cervical vertebrae of the vertebral column...

; CACHD1; CACNA2D1

CACNA2D1

Voltage-dependent calcium channel subunit alpha-2/delta-1 is a protein that in humans is encoded by the CACNA2D1 gene.- External links :...

; CACNA2D2

CACNA2D2

Voltage-dependent calcium channel subunit alpha2delta-2 is a protein that in humans is encoded by the CACNA2D2 gene....

; CACNA2D3;
CACNA2D4; CFB; CLCA1

CLCA1

Calcium-activated chloride channel regulator 1 is a protein that in humans is encoded by the CLCA1 gene....

; CLCA2; CLCA4

CLCA4

Chloride channel, calcium activated, family member 4, also known as CLCA4, is a protein which in humans CLCA4 gene. The protein encoded by this gene is a chloride channel. Protein structure prediction methods suggest the N-terminal region of CLCA4 protein is a zinc metalloprotease, and the protein...

; COCH

COCH

Cochlin is a protein that in humans is encoded by the COCH gene.-Further reading:...

; COL12A1

COL12A1

Collagen alpha-1 chain is a protein that in humans is encoded by the COL12A1 gene.-Further reading:...

; COL14A1

COL14A1

Collagen alpha-1 chain is a protein that in humans is encoded by the COL14A1 gene.-Further reading:...

;
COL20A1

COL20A1

COL20A1 is a collagen gene....

; COL21A1

COL21A1

COL21A1 is a collagen gene....

; COL22A1

COL22A1

COL22A1 is a collagen gene....

; COL28; COL6A1

COL6A1

Collagen alpha-1 chain is a protein that in humans is encoded by the COL6A1 gene.- Function :The collagens are a superfamily of proteins that play a role in maintaining the integrity of various tissues. Collagens are extracellular matrix proteins and have a triple-helical domain as their common...

; COL6A2

COL6A2

Collagen alpha-2 chain is a protein that in humans is encoded by the COL6A2 gene.- Function :This gene encodes one of the three alpha chains of type VI collagen, a beaded filament collagen found in most connective tissues. The product of this gene contains several domains similar to von Willebrand...

; COL6A3

COL6A3

Collagen alpha-3 chain is a protein that in humans is encoded by the COL6A3 gene.- Function :This gene encodes the alpha 3 chain, one of the three alpha chains of type VI collagen, a beaded filament collagen found in most connective tissues. The alpha 3 chain of type VI collagen is much larger than...

; COL7A1

COL7A1

Collagen alpha-1 chain is a protein that in humans is encoded by the COL7A1 gene.- Function :This gene encodes the alpha chain of type VII collagen. The type VII collagen fibril, composed of three identical alpha collagen chains, is restricted to the basement zone beneath stratified squamous...

;
COLA1L; CaCC1; ITGA1; ITGA10

ITGA10

Integrin alpha-10 also known as ITGA10 is a protein that in humans is encoded by the ITGA10 gene.-Function:Integrins are integral membrane proteins composed of an alpha chain and a beta chain, and are known to participate in cell adhesion as well as cell-surface mediated signalling...

; ITGA11

ITGA11

Integrin alpha-11 is a protein that in humans is encoded by the ITGA11 gene.This gene encodes an alpha integrin. Integrins are heterodimeric integral membrane proteins composed of an alpha chain and a beta chain. This protein contains an I domain, is expressed in muscle tissue, dimerizes with beta...

; ITGA2; ITGAD

ITGAD

Integrin alpha-D is a protein that in humans is encoded by the ITGAD gene.-External links:* Info with links in the...

; ITGAE

ITGAE

Integrin, alpha E also known as CD103 is an integrin protein that in human is encoded by the ITGAE gene. CD103 binds integrin beta 7 to form the complete heterodimeric integrin molecule αEβ7, which has no distinct name...

;
ITGAL; ITGAM; ITGAX; ITIH1

ITIH1

Inter-alpha-trypsin inhibitor heavy chain H1 is a protein that in humans is encoded by the ITIH1 gene....

; ITIH2

ITIH2

Inter-alpha-trypsin inhibitor heavy chain H2 is a protein that in humans is encoded by the ITIH2 gene....

; ITIH3

ITIH3

Inter-alpha-trypsin inhibitor heavy chain H3 is a protein that in humans is encoded by the ITIH3 gene....

; ITIH4

ITIH4

Inter-alpha-trypsin inhibitor heavy chain H4 is a protein that in humans is encoded by the ITIH4 gene....

; ITIH5;
ITIH5L; LOC285929; LOC340267; LOC389462; LOH11CR2A; MATN1

MATN1

Matrilin 1, cartilage matrix protein, also known as MATN1, is a protein which in humans is encoded by the MATN1 gene.- Function :This gene encodes a member of von Willebrand factor A domain containing protein family. This family of proteins are thought to be involved in the formation of filamentous...

; MATN2

MATN2

Matrilin-2 is a protein that in humans is encoded by the MATN2 gene.-Further reading:...

; MATN3

MATN3

Matrilin-3 is a protein that in humans is encoded by the MATN3 gene.-External links:* -Further reading:...

;
MATN4; PARP4

PARP4

Poly [ADP-ribose] polymerase 4 is an enzyme that in humans is encoded by the PARP4 gene.-Interactions:PARP4 has been shown to interact with Major vault protein.-Further reading:...

; SEL-OB; SVEP1; VIT

VIT

VIT may refer to:* VIT University* VIT, C.A., a computer hardware manufacturer* Venkateshwara Institute of Technology* Victorian Institute of Teaching* Vidyalankar Institute of Technology* Vishnu Institute of Technology...

; VWA1; VWA2

VWA2

von Willebrand factor A domain-containing protein 2, also known as A domain-containing protein similar to matrilin and collagen is a protein that in humans is encoded by the VWA2 gene....

; VWF

Von Willebrand factor

von Willebrand factor is a blood glycoprotein involved in hemostasis. It is deficient or defective in von Willebrand disease and is involved in a large number of other diseases, including thrombotic thrombocytopenic purpura, Heyde's syndrome, and possibly hemolytic-uremic syndrome...

;
hCLCA1; hCLCA2;

External Links