title: Binding of the von Hippel-Lindau tumor suppressor protein to Elongin B and C.
abstract: Germ-line mutations of the von Hippel-Lindau tumor suppressor gene (VHL) predispose individuals to a variety of human tumors, and somatic mutations of this gene have been identified in sporadic renal cell carcinomas and cerebellar hemangioblastomas. Two transcriptional elongation factors, Elongin B and C, were shown to bind in vitro and in vivo to a short, colinear region of the VHL protein (pVHL) that is frequently mutated in human tumors. A peptide replica of this region inhibited binding of pVHL to Elongin B and C whereas a point-mutant derivative, corresponding to a naturally occurring VHL missense mutation, had no effect. These results suggest that the tumor suppression function of pVHL may be linked to its ability to bind to Elongin B and C.
authors.affiliation: Dana-Farber Cancer Institute, Boston, MA 02115, USA.
authors.forename: A
authors.initials: A
authors.lastname: Kibel
authors.forename: O
authors.initials: O
authors.lastname: Iliopoulos
authors.forename: J A
authors.initials: JA
authors.lastname: DeCaprio
authors.forename: W G
authors.initials: WG
authors.lastname: Kaelin
mesh_terms: D000595:Amino Acid Sequence; D000818:Animals; D002292:Carcinoma, Renal Cell; D000075065:Elongin; D016147:Genes, Tumor Suppressor; D018095:Germ-Line Mutation; D006801:Humans; D008025:Ligases; D051379:Mice; D008969:Molecular Sequence Data; D009687:Nuclear Proteins / Q000235:genetics / Q000378:metabolism*; D017354:Point Mutation; D011993:Recombinant Fusion Proteins / Q000378:metabolism; D014157:Transcription Factors / Q000378:metabolism*; D014162:Transfection; D014407:Tumor Cells, Cultured; D025521:Tumor Suppressor Proteins; D044767:Ubiquitin-Protein Ligases; D051794:Von Hippel-Lindau Tumor Suppressor Protein; D006623:von Hippel-Lindau Disease / Q000235:genetics
chemical_list: C000616396:ELOB protein, human; C000616401:Elob protein, mouse; D000075065:Elongin; D009687:Nuclear Proteins; D011993:Recombinant Fusion Proteins; D014157:Transcription Factors; D025521:Tumor Suppressor Proteins; D044767:Ubiquitin-Protein Ligases; D051794:Von Hippel-Lindau Tumor Suppressor Protein; D008025:Ligases; C497446:VHL protein, human