Erythrocytosis: the HIF pathway in control

K Franke, M Gassmann… - Blood, The Journal of the …, 2013 - ashpublications.org
Organisms living under aerobic conditions need oxygen for the metabolic conversion of
nutrition into energy. With the appearance of increasingly complex animals, a specialized …

The HIF pathway and erythrocytosis

FS Lee, MJ Percy - Annual Review of Pathology: Mechanisms of …, 2011 - annualreviews.org
Because of the central role that red blood cells play in the delivery of oxygen to tissues of the
body, red blood cell mass must be controlled at precise levels. The glycoprotein hormone …

Involvement of oxygen-sensing pathways in physiologic and pathologic erythropoiesis

GL Semenza - Blood, The Journal of the American Society of …, 2009 - ashpublications.org
Red blood cells deliver O2 from the lungs to every cell in the human body. Reduced tissue
oxygenation triggers increased production of erythropoietin by hypoxia-inducible factor 1 …

A family with erythrocytosis establishes a role for prolyl hydroxylase domain protein 2 in oxygen homeostasis

MJ Percy, Q Zhao, A Flores… - Proceedings of the …, 2006 - National Acad Sciences
The number of red blood cells is normally tightly regulated by a classic homeostatic
mechanism based on oxygen sensing in the kidney. Decreased oxygen delivery resulting …

Update on mutations in the HIF: EPO pathway and their role in erythrocytosis

TR Lappin, FS Lee - Blood reviews, 2019 - Elsevier
Identification of the underlying defects in congenital erythrocytosis has provided mechanistic
insights into the regulation of erythropoiesis and oxygen homeostasis. The Hypoxia …

[HTML][HTML] Regulation of erythropoiesis by the hypoxia-inducible factor pathway: effects of genetic and pharmacological perturbations

GL Semenza - Annual review of medicine, 2023 - annualreviews.org
Red blood cells transport O2 from the lungs to body tissues. Hypoxia stimulates kidney cells
to secrete erythropoietin (EPO), which increases red cell mass. Hypoxia-inducible factors …

Regulation of erythropoiesis by hypoxia-inducible factors

VH Haase - Blood reviews, 2013 - Elsevier
A classic physiologic response to systemic hypoxia is the increase in red blood cell
production. Hypoxia-inducible factors (HIFs) orchestrate this response by inducing cell-type …

[HTML][HTML] Erythrocytosis and pulmonary hypertension in a mouse model of human HIF2A gain of function mutation

Q Tan, H Kerestes, MJ Percy, R Pietrofesa… - Journal of Biological …, 2013 - ASBMB
The central pathway for oxygen-dependent control of red cell mass is the prolyl hydroxylase
domain protein (PHD): hypoxia inducible factor (HIF) pathway. PHD site specifically prolyl …

[HTML][HTML] Erythrocytosis-associated HIF-2α mutations demonstrate a critical role for residues C-terminal to the hydroxylacceptor proline

PW Furlow, MJ Percy, S Sutherland, C Bierl… - Journal of Biological …, 2009 - ASBMB
A classic physiologic response to hypoxia in humans is the up-regulation of the
ERYTHROPOIETIN (EPO) gene, which is the central regulator of red blood cell mass. The …

Regulation of adult erythropoiesis by prolyl hydroxylase domain proteins

K Takeda, HL Aguila, NS Parikh, X Li… - Blood, The Journal …, 2008 - ashpublications.org
Polycythemia is often associated with erythropoietin (EPO) overexpression and defective
oxygen sensing. In normal cells, intracellular oxygen concentrations are directly sensed by p …