PF00092

von Willebrand factor type A domain

Pfam entry
Member databasePfam
Pfam typedomain
Short nameVWA
ClanvWA-like
Author Sonnhammer ELL;0000-0002-9015-5588 Bateman A;0000-0002-6982-4660
Sequence Ontology0000417

Description
Imported from IPR002035

The von Willebrand factor is a large multimeric glycoprotein found in blood plasma. Mutant forms are involved in the aetiology of bleeding disorders
[2]
. 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
[3, 4, 1]
. 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. 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
[3]
. A number of human diseases arise from mutations in VWA domains.

References
Imported from IPR002035

1.Shuffled domains in extracellular proteins. Bork P. FEBS Lett. 286, 47-54, (1991). View articlePMID: 1864378

2.von Willebrand factor. Ruggeri ZM, Ware J. FASEB J. 7, 308-16, (1993). View articlePMID: 8440408

3.Type A modules: interacting domains found in several non-fibrillar collagens and in other extracellular matrix proteins. Colombatti A, Bonaldo P, Doliana R. Matrix 13, 297-306, (1993). PMID: 8412987

4.The secondary structure of the von Willebrand factor type A domain in factor B of human complement by Fourier transform infrared spectroscopy. Its occurrence in collagen types VI, VII, XII and XIV, the integrins and other proteins by averaged structure predictions. Perkins SJ, Smith KF, Williams SC, Haris PI, Chapman D, Sim RB. J. Mol. Biol. 238, 104-19, (1994). View articlePMID: 8145250

Further reading

5. The protein fold of the von Willebrand factor type A domain is predicted to be similar to the open twisted beta-sheet flanked by alpha-helices found in human ras-p21. Edwards YJ, Perkins SJ. FEBS Lett. 358, 283-6, (1995). View articlePMID: 7843416

6. Crystal structure of the A domain from the alpha subunit of integrin CR3 (CD11b/CD18). Lee JO, Rieu P, Arnaout MA, Liddington R. Cell 80, 631-8, (1995). View articlePMID: 7867070

7. Crystal structure of the I-domain from the CD11a/CD18 (LFA-1, alpha L beta 2) integrin. Qu A, Leahy DJ. Proc. Natl. Acad. Sci. U.S.A. 92, 10277-81, (1995). View articlePMID: 7479767

Wikipedia

This website requires cookies, and the limited processing of your personal data in order to function. By using the site you are agreeing to this as outlined in our Privacy Notice and Terms of Use.