von Hippel-Lindau (VHL) disease is a hereditary cancer with a predilection for the central nervous system and retina. The von Hippel-Lindau tumor suppressor gene is mutated in families with von Hippel-Lindau disease and encodes a protein (VHL) of 213 amino acids with an acidic pentapeptide motif in the N-terminus. Mutations in the VHL gene result in constitutive expression of many hypoxi-induced genes, at least in part because of increases in the cellular level of hypoxia-inducible transcription factor HIF-1α. VHL protein binds to elongin B, elongin C, and Cul2 to form a stable complex that targets hypoxia inducibal factors (HIFs) for degradation and transcriptional regulation. In addition, VHL protein has also been shown to interact with specific protein kinase C isoforms, histone deacetylases and HIF-1 inhibitor (FIH-1). α-VHL is an affinity-purified rabbit polyclonal antibody raised against the recombinant VHL protein.
Intended for research use only. Not for use in human, therapeutic, or diagnostic applications.