Fig. 8: Schematic of oxygen-dependent regulation of mitochondrial content within the von Hippel–Lindau syndrome.

a, Genotype–phenotype correlation in cancers arising in the VHL syndrome and its association with regulation of HIFα and mitochondrial content. Note that the Cuvash polycythaemia mutation VHLR200W shows total absence of tumour development despite increased HIFα signalling and appears normal with regard to regulating mitochondrial content. b, Schematic of oxygen-dependent regulation of mitochondrial transcription factor TFAM by pVHL, independent of the canonical substrate HIFα. mtDNA, mitochondrial DNA.