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  • Title: Binding of the von Hippel-Lindau tumor suppressor protein to Elongin B and C.
    Author: Kibel A, Iliopoulos O, DeCaprio JA, Kaelin WG.
    Journal: Science; 1995 Sep 08; 269(5229):1444-6. PubMed ID: 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.
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