von Hippel-Lindau (VHL) Protein

R. C. Conaway, J. W. Conaway

Research output: Chapter in Book/Report/Conference proceedingChapter


The von Hippel-Lindau (VHL) protein is a tumor suppressor. Mutations in the VHL protein can give rise to tumors of multiple organ systems, including the central nervous system, the endocrine system, and the kidney. The VHL protein functions as a subunit of a multiprotein ubiquitin ligase that negatively regulates expression of a large collection of hypoxia-inducible genes controlled by hypoxia-inducible transcription factors (HIFs). The VHL ubiquitin ligase prevents inappropriate expression of these hypoxia-inducible genes when cells are grown in a plentiful supply of oxygen by targeting HIFs for rapid ubiquitylation and degradation by the proteasome.

Original languageEnglish (US)
Title of host publicationEncyclopedia of Biological Chemistry
Subtitle of host publicationSecond Edition
PublisherElsevier Inc.
Number of pages2
ISBN (Electronic)9780123786319
ISBN (Print)9780123786302
StatePublished - Feb 15 2013
Externally publishedYes


  • Cullin protein
  • Dioxygenase
  • Elongin B
  • Elongin C
  • HIF
  • Hypoxia
  • Prolyl hydroxylation
  • Renal carcinoma
  • Ring finger protein
  • Transcription
  • Ubiquitin
  • VEGF
  • VHL

ASJC Scopus subject areas

  • Biochemistry, Genetics and Molecular Biology(all)


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