pVHL: a multipurpose adaptor protein.
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.
Institute of Cell Biology, Eidgenössische Technische Hochschule Zurich, 8093 Zurich, Switzerland.
Ian J
IJ
Frew
Wilhelm
W
Krek
D048868:Adaptor Proteins, Signal Transducing / Q000378:metabolism*; D002352:Carrier Proteins / Q000378:metabolism*; D003599:Cytoskeleton / Q000502:physiology; D005109:Extracellular Matrix / Q000502:physiology; D005786:Gene Expression Regulation; D006706:Homeostasis; D006801:Humans; D008870:Microtubules / Q000502:physiology; D012097:Repressor Proteins / Q000378:metabolism*; D014158:Transcription, Genetic; D051794:Von Hippel-Lindau Tumor Suppressor Protein / Q000235:genetics*; D006623:von Hippel-Lindau Disease / Q000235:genetics
D048868:Adaptor Proteins, Signal Transducing; D002352:Carrier Proteins; D012097:Repressor Proteins; D051794:Von Hippel-Lindau Tumor Suppressor Protein; C497446:VHL protein, human