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Many adaptive responses to hypoxia involve changes in gene transcription mediated by the hypoxia-inducible factor 1 complex. Central to this is oxygen-dependent proteolysis of the alpha subunit, which has recently been shown to require the von Hippel-Lindau tumour-suppressor protein. This observation provides one mechanism by which inherited defects in the von Hippel-Lindau gene could cause features of the clinical syndrome, and offers insight into the events leading to sporadic clear cell renal cancer. Furthermore, it clearly implicates the von Hippel-Lindau tumour-suppressor protein in the biochemistry of oxygen sensing.

Original publication

DOI

10.1159/000052617

Type

Journal article

Journal

Exp Nephrol

Publication Date

2001

Volume

9

Pages

235 - 240

Keywords

DNA-Binding Proteins, Genes, Tumor Suppressor, Helix-Loop-Helix Motifs, Humans, Hypoxia, Hypoxia-Inducible Factor 1, Hypoxia-Inducible Factor 1, alpha Subunit, Nuclear Proteins, Transcription Factors, von Hippel-Lindau Disease