| First Author | Lenoir A | Year | 2011 |
| Journal | Blood | Volume | 117 |
| Issue | 2 | Pages | 647-50 |
| PubMed ID | 20940420 | Mgi Jnum | J:168426 |
| Mgi Id | MGI:4888188 | Doi | 10.1182/blood-2010-07-295147 |
| Citation | Lenoir A, et al. (2011) Iron-deficiency anemia from matriptase-2 inactivation is dependent on the presence of functional Bmp6. Blood 117(2):647-50 |
| abstractText | 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. |
