Afficher la couverture 'Iron-deficiency anemia from matriptase-2 inactivation is dependent on the presence of functional Bmp6.. Iron-deficiency anemia from matriptase-2 inactivation is dependent on the presence of functional Bmp6.: Matriptase-2 and Bmp6 deficiency in mice | Lenoir, Anne' en grand format
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Iron-deficiency anemia from matriptase-2 inactivation is dependent on the presence of functional Bmp6.. Iron-deficiency anemia from matriptase-2 inactivation is dependent on the presence of functional Bmp6.: Matriptase-2 and Bmp6 deficiency in mice

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Lenoir, Anne | Deschemin, Jean-Christophe | Kautz, Léon | Ramsay, Andrew, J. | Roth, Marie-Paule | Lopez-Otin, Carlos | Vaulont, Sophie | Nicolas, Gaël

Edité par CCSD ; American Society of Hematology -

International audience. Hepcidin is the master regulator of iron homeostasis. In the liver, iron-dependent hepcidin activation is regulated through Bmp6 and its membrane receptor hemojuvelin (Hjv), whereas, in response to iron deficiency, hepcidin repression seems to be controlled by a pathway involving the serine protease matriptase-2 (encoded by Tmprss6). To determine the relationship between Bmp6 and matriptase-2 pathways, Tmprss6(-/-) mice (characterized by increased hepcidin levels and anemia) and Bmp6(-/-) mice (exhibiting severe iron overload because of hepcidin deficiency) were intercrossed. We showed that loss of Bmp6 decreased hepcidin levels; increased hepatic iron; and, importantly, corrected hematologic abnormalities in Tmprss6(-/-) mice. This finding suggests that elevated hepcidin levels in patients with familial iron-refractory, iron-deficiency anemia are the result of excess signaling through the Bmp6/Hjv pathway.

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