Insights into the role of the von Hippel-Lindau gene product. A key player in hypoxic regulation.

Journal article

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.

Authors: Maxwell, P; Pugh, C; Ratcliffe, P

• Publication status: Published
• Journal: Experimental nephrology
• Volume: 9
• Issue: 4
• Pages: 235-240
• Publication date: 2001-01-01
• ISSN: 1018-7782
• Language: English
• Keywords: Hypoxia-Inducible Factor 1; Anoxia; Humans; Transcription Factors; DNA-Binding Proteins; Helix-Loop-Helix Motifs; Nuclear Proteins; von Hippel-Lindau Disease; Genes, Tumor Suppressor; Hypoxia-Inducible Factor 1, alpha Subunit