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Sci. Signal., 17 June 2008
Vol. 1, Issue 24, p. pe30
[DOI: 10.1126/scisignal.124pe30]


pVHL: A Multipurpose Adaptor Protein

Ian J. Frew and Wilhelm Krek*

Institute of Cell Biology, Eidgenössische Technische Hochschule (ETH) Zurich, 8093 Zurich, Switzerland.

Abstract: Mutations in the von Hippel-Lindau tumor suppressor gene VHL occur in various inherited and sporadically occurring tumors. The protein encoded by VHL—pVHL—bears no known enzymatic activities but interacts with numerous protein partners. With the identification of distinct pVHL-containing multiprotein complexes, a refined portrait of pVHL tumor suppressor function has arisen. In general, pVHL acts as a multipurpose adaptor protein that controls a diverse array of gene expression programs, as well as extracellular matrix assembly and microtubule-based processes, by linking various target proteins to appropriate enzymatic activities. These findings provide an evermore complex but coherent view of how pVHL functions molecularly and of the consequences of dysregulation of these diverse molecular activities on tumor formation.

*Corresponding author. Institute of Cell Biology, ETH Zurich, 8093 Zurich, Switzerland. Telephone, +41 44 633 3447; fax, +41 44 633 1357; e-mail, wilhelm.krek{at}

Citation: I. J. Frew, W. Krek, pVHL: A Multipurpose Adaptor Protein. Sci. Signal. 1, pe30 (2008).

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