ABSTRACT
Oxygen is a central molecule in the development of multicellular life, allowing efficient energy generation. Inadequate oxygen supply requires rapid adaptations to prevent cellular damage and the hypoxia-inducible factor (HIF) pathway plays a central role in this adaptation. Numerous diseases and disease processes are influenced by hypoxia and the HIF pathway. One component, von Hippel Lindau (VHL), is a well-known tumor suppressor, which acts at least in part via regulating HIF signaling. The zebrafish has become a central vertebrate model organism in which developmental and disease processes can be studied. In this review, we have tried to bring together knowledge on the HIF/hypoxic signaling pathway in zebrafish, including what is known on VHL functions.