Iron metabolism and ineffective erythropoiesis in beta-thalassemia mouse models.
beta-thalassemia is a disease associated with decreased beta-globin production leading to anemia, ineffective erythropoiesis, and iron overload. New mechanisms associated with modulation of erythropoiesis and iron metabolism have recently been discovered in thalassemic mice, improving our understanding of the pathophysiology of this disease. These discoveries have the potential to be translated into clinically-relevant therapeutic options to reduce ineffective erythropoiesis and iron overload. A new generation of therapies based on limiting ineffective erythropoiesis, iron absorption, and the correction of iron maldistribution could be on the way, possibly complementing and improving the current standard of patient care.
Ramos
Pedro
P
Division of Hematology-Oncology, Department of Pediatrics, Children's Blood Foundation Laboratories, Weill Medical College of Cornell University, New York, New York, USA.
Melchiori
Luca
L
Gardenghi
Sara
S
Van-Roijen
Nico
N
Grady
Robert W
RW
Ginzburg
Yelena
Y
Rivella
Stefano
S
D000818:Animals; D000900:Anti-Bacterial Agents / Q000378:metabolism; D023181:Antimicrobial Cationic Peptides / Q000378:metabolism; D004195:Disease Models, Animal; D004920:Erythropoiesis / Q000502:physiology*; D064451:Hepcidins; D006801:Humans; D007501:Iron / Q000378:metabolism*; D019190:Iron Overload / Q000378:metabolism; D051379:Mice; D017086:beta-Thalassemia / Q000378:metabolism* / Q000503:physiopathology*
D000900:Anti-Bacterial Agents; D023181:Antimicrobial Cationic Peptides; D064451:Hepcidins; D007501:Iron