Binding of the von Hippel-Lindau Tumor Suppressor Protein to Elongin B and C
- 8 September 1995
- journal article
- other
- Published by American Association for the Advancement of Science (AAAS) in Science
- Vol. 269 (5229), 1444-1446
- https://doi.org/10.1126/science.7660130
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.Keywords
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