Skip to main content
. Author manuscript; available in PMC: 2022 Jun 29.
Published in final edited form as: Nat Metab. 2022 Jun 27;4(6):739–758. doi: 10.1038/s42255-022-00593-x

Figure 8. Schematic of oxygen dependent regulation of mitochondrial content within the von Hippel-Lindau (VHL) syndrome.

Figure 8

a, Genotype-phenotype correlation in cancers arising in the VHL syndrome and its association with regulation of HIFα and mitochondrial content. Note: Cuvash polycythemia mutation VHLR200W shows total absence of tumor development despite increased HIFα signaling and appears normal in regard of regulating mitochondrial content. b, Schematic representation of oxygen dependent regulation of mitochondrial transcription factor TFAM by pVHL, independent of the canonical substrate HIFα.