Publication Date:
1995-09-08
Description:
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.〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Kibel, A -- Iliopoulos, O -- DeCaprio, J A -- Kaelin, W G Jr -- New York, N.Y. -- Science. 1995 Sep 8;269(5229):1444-6.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Dana-Farber Cancer Institute, Boston, MA 02115, USA.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/7660130" target="_blank"〉PubMed〈/a〉
Keywords:
Amino Acid Sequence
;
Animals
;
Carcinoma, Renal Cell
;
*Genes, Tumor Suppressor
;
Germ-Line Mutation
;
Humans
;
*Ligases
;
Mice
;
Molecular Sequence Data
;
Nuclear Proteins/genetics/*metabolism
;
Point Mutation
;
Recombinant Fusion Proteins/metabolism
;
Transcription Factors/*metabolism
;
Transfection
;
Tumor Cells, Cultured
;
*Tumor Suppressor Proteins
;
*Ubiquitin-Protein Ligases
;
Von Hippel-Lindau Tumor Suppressor Protein
;
von Hippel-Lindau Disease/genetics
Print ISSN:
0036-8075
Electronic ISSN:
1095-9203
Topics:
Biology
,
Chemistry and Pharmacology
,
Computer Science
,
Medicine
,
Natural Sciences in General
,
Physics
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