Thalassaemia

A Kattamis, JL Kwiatkowski, Y Aydinok - The lancet, 2022 - thelancet.com
Thalassaemia is a diverse group of genetic disorders with a worldwide distribution affecting
globin chain synthesis. The pathogenesis of thalassaemia lies in the unbalanced globin …

Thalassaemia

AT Taher, DJ Weatherall, MD Cappellini - The Lancet, 2018 - thelancet.com
Inherited haemoglobin disorders, including thalassaemia and sickle-cell disease, are the
most common monogenic diseases worldwide. Several clinical forms of α-thalassaemia and …

[HTML][HTML] A red carpet for iron metabolism

MU Muckenthaler, S Rivella, MW Hentze, B Galy - Cell, 2017 - cell.com
200 billion red blood cells (RBCs) are produced every day, requiring more than 2× 10 15
iron atoms every second to maintain adequate erythropoiesis. These numbers translate into …

Identification of erythroferrone as an erythroid regulator of iron metabolism

L Kautz, G Jung, EV Valore, S Rivella, E Nemeth… - Nature …, 2014 - nature.com
Recovery from blood loss requires a greatly enhanced supply of iron to support expanded
erythropoiesis. After hemorrhage, suppression of the iron-regulatory hormone hepcidin …

Iron as a central player and promising target in cancer progression

M Jung, C Mertens, E Tomat, B Brüne - International journal of molecular …, 2019 - mdpi.com
Iron is an essential element for virtually all organisms. On the one hand, it facilitates cell
proliferation and growth. On the other hand, iron may be detrimental due to its redox …

Systemic iron homeostasis

T Ganz - Physiological reviews, 2013 - journals.physiology.org
The iron hormone hepcidin and its receptor and cellular iron exporter ferroportin control the
major fluxes of iron into blood plasma: intestinal iron absorption, the delivery of recycled iron …

Iron overload in thalassemia: different organs at different rates

AT Taher, AN Saliba - Hematology 2014, the American Society …, 2017 - ashpublications.org
Thalassemic disorders lie on a phenotypic spectrum of clinical severity that depends on the
severity of the globin gene mutation and coinheritance of other genetic determinants. Iron …

[HTML][HTML] Hepcidin and iron homeostasis

T Ganz, E Nemeth - Biochimica et Biophysica Acta (BBA)-Molecular Cell …, 2012 - Elsevier
Despite fluctuations in dietary iron intake and intermittent losses through bleeding, the
plasma iron concentrations in humans remain stable at 10–30μM. While most of the iron …

Hepcidin and the iron-infection axis

H Drakesmith, AM Prentice - science, 2012 - science.org
Iron lies at the center of a battle for nutritional resource between higher organisms and their
microbial pathogens. The iron status of the human host affects the pathogenicity of …

Hepcidin and iron regulation, 10 years later

T Ganz - Blood, The Journal of the American Society of …, 2011 - ashpublications.org
Under evolutionary pressure to counter the toxicity of iron and to maintain adequate iron
supply for hemoglobin synthesis and essential metabolic functions, humans and other …