Physiological and pathophysiological mechanisms of hepcidin regulation: clinical implications for iron disorders.
Br J Haematol
; 193(5): 882-893, 2021 06.
Article
em En
| MEDLINE
| ID: mdl-33316086
ABSTRACT
The discovery of hepcidin has provided a solid foundation for understanding the mechanisms of systemic iron homeostasis and the aetiologies of iron disorders. Hepcidin assures the balance of circulating and stored iron levels for multiple physiological processes including oxygen transport and erythropoiesis, while limiting the toxicity of excess iron. The liver is the major site where regulatory signals from iron, erythropoietic drive and inflammation are integrated to control hepcidin production. Pathologically, hepcidin dysregulation by genetic inactivation, ineffective erythropoiesis, or inflammation leads to diseases of iron deficiency or overload such as iron-refractory iron-deficiency anaemia, anaemia of inflammation, iron-loading anaemias and hereditary haemochromatosis. In the present review, we discuss recent insights into the molecular mechanisms governing hepcidin regulation, how these pathways are disrupted in iron disorders, and how this knowledge is being used to develop novel diagnostic and therapeutic strategies.
Palavras-chave
Texto completo:
1
Coleções:
01-internacional
Temas:
Agentes_cancerigenos
Base de dados:
MEDLINE
Assunto principal:
Anemia Ferropriva
/
Eritropoese
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Hepcidinas
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Hemocromatose
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Fígado
Limite:
Animals
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Humans
Idioma:
En
Revista:
Br J Haematol
Ano de publicação:
2021
Tipo de documento:
Article
País de afiliação:
Estados Unidos