Erythrocytosis: the HIF pathway in control

被引：120

作者：

Franke, Kristin ^{[1
]}

Gassmann, Max ^{[2
,3
,4
]}

Wielockx, Ben ^{[1
,5
,6
]}

机构：

[1] Tech Univ Dresden, Emmy Noether Res Grp, Inst Pathol, Dresden, Germany

[2] Vetsuisse Fac, Inst Vet Physiol, Zurich, Switzerland

[3] Zurich Ctr Integrat Human Physiol ZIHP, Zurich, Switzerland

[4] Univ Peruana Cayetano Heredia, Lima, Peru

[5] Tech Univ Dresden, DFG Res Ctr, Dresden, Germany

[6] Tech Univ Dresden, Cluster Excellence Regenerat Therapies Dresden, Dresden, Germany

来源：

BLOOD | 2013年 / 122卷 / 07期

基金：

瑞士国家科学基金会;

关键词：

HYPOXIA-INDUCIBLE-FACTOR; HYDROXYLASE DOMAIN PROTEIN-2; ERYTHROPOIETIN-PRODUCING CELLS; CHUVASH POLYCYTHEMIA; CONGENITAL POLYCYTHEMIA; VHL MUTATION; STEM-CELL; VASCULAR TUMORS; MESSENGER-RNA; PHD2; MUTATION;

D O I：

10.1182/blood-2013-01-478065

中图分类号：

R5 [内科学];

学科分类号：

100201 [内科学];

摘要：

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, HIF2a, VHL) lead to disorders in blood homeostasis as a result of insufficient (anemia) or excessive (erythrocytosis) red blood cell production.

引用

页码：1122 / 1128

页数：7

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