Erythrocytosis: the HIF pathway in control

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  • Kristin Franke
    Emmy Noether Research Group, Institute of Pathology, University of Technology, Dresden, Germany;
  • Max Gassmann
    Institute of Veterinary Physiology, Vetsuisse Faculty and Zurich Center for Integrative Human Physiology (ZIHP), Zurich, Switzerland;
  • Ben Wielockx
    Emmy Noether Research Group, Institute of Pathology, University of Technology, Dresden, Germany;

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<jats:title>Abstract</jats:title><jats:p>Organisms living under aerobic conditions need oxygen for the metabolic conversion of nutrition into energy. With the appearance of increasingly complex animals, a specialized transport system (erythrocytes) arose during evolution to provide oxygen to virtually every single cell in the body. Moreover, in case of low environmental partial pressure of oxygen, the number of erythrocytes automatically increases to preserve sustained oxygen delivery. This process relies predominantly on the cytokine erythropoietin (Epo) and its transcription factor hypoxia inducible factor (HIF), whereas the von Hippel-Lindau (VHL) ubiquitin ligase as well as the oxygen-sensitive prolyl hydroxylases (PHDs) represent essential regulators of this oxygen-sensing system. Deregulation of particular members of this pathway (eg, PHD2, HIF2α, VHL) lead to disorders in blood homeostasis as a result of insufficient (anemia) or excessive (erythrocytosis) red blood cell production.</jats:p>

Journal

  • Blood

    Blood 122 (7), 1122-1128, 2013-08-15

    American Society of Hematology

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