Hypoxia-inducible factor 2α regulates expression of the mitochondrial aconitase chaperone protein frataxin

Y Oktay, E Dioum, S Matsuzaki, K Ding, LJ Yan… - Journal of biological …, 2007 - ASBMB
Mice lacking Epas1, encoding the transcription factor Hypoxia-inducible Factor 2α (HIF-2α),
exhibit an apparent mitochondrial disease state. Similarities between knock-outs of Epas1
and of Sod2, encoding the mitochondrial antioxidant enzyme manganese superoxide
dismutase, led to the identification of Sod2 as a HIF-2α target gene. However, Sod2 levels in
Epas1–/–liver are intermediate between that of Sod+/–and Sod2–/–mice, which have subtle
or severe phenotypes, respectively. This suggests that additional HIF-2α target genes …