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.
journal_name
Bloodjournal_title
Bloodauthors
Lenoir A,Deschemin JC,Kautz L,Ramsay AJ,Roth MP,Lopez-Otin C,Vaulont S,Nicolas Gdoi
10.1182/blood-2010-07-295147subject
Has Abstractpub_date
2011-01-13 00:00:00pages
647-50issue
2eissn
0006-4971issn
1528-0020pii
blood-2010-07-295147journal_volume
117pub_type
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