Maxwell P H, Pugh C W, Ratcliffe P J
Nephrology and Oxygen-Sensing Laboratory, Henry Wellcome Building of Genomic Medicine, Oxford, UK.
Exp Nephrol. 2001;9(4):235-40. doi: 10.1159/000052617.
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.
