H
IPR036465

von Willebrand factor A-like domain superfamily

InterPro entry
Short namevWFA_dom_sf
Overlapping entries
 
Ssl1-like (IPR007198)

Description

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

References

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

2.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

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

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. 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

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. 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

Cross References

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