Iron-deficiency anemia from matriptase-2 inactivation is dependent on the presence of functional Bmp6.: Matriptase-2 and Bmp6 deficiency in mice

Abstract : 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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Blood, American Society of Hematology, 2011, 117 (2), pp.647-50. 〈10.1182/blood-2010-07-295147〉
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Anne Lenoir, Jean-Christophe Deschemin, Léon Kautz, Andrew Ramsay, Marie-Paule Roth, et al.. Iron-deficiency anemia from matriptase-2 inactivation is dependent on the presence of functional Bmp6.: Matriptase-2 and Bmp6 deficiency in mice. Blood, American Society of Hematology, 2011, 117 (2), pp.647-50. 〈10.1182/blood-2010-07-295147〉. 〈inserm-00552073〉

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