Hypoxia-Inducible Factor 2α: at the Interface between Oxygen Sensing Systems in Physiology and Pathology

More than 100 years after the original descriptions of altitude adaptation, it is now clear that many of these responses are mediated by a specific isoform of the transcription factor hypoxia-inducible factor (HIF)-2α. Here, we review this work, including connectivity with the oxygen chemosensitive response itself and with paraganglioma, a tumor often affecting chemosensitive tissues.