Abstract:
BACKGROUND:Impaired regulation of hepcidin in response to iron is the cause of genetic hemochromatosis associated with defects of HFE and transferrin receptor 2. However, the role of these proteins in the regulation of hepcidin expression is unclear. DESIGN AND METHODS:Hepcidin expression, SMAD and extracellular signal-regulated kinase (Erk) phosphorylation and furin expression were analyzed in hepatic HepG2 cells in which HFE and transferrin receptor 2 were down-regulated or expressed, or furin activity specifically inhibited. Furin expression was also analyzed in the liver of transferrin receptor 2 null mice. RESULTS:We showed that the silencing of HFE and transferrin receptor 2 reduced both Erk phosphorylation and furin expression, that the exogenous expression of the two enhanced the induction of phosphoErk1/2 and furin by holotransferrin, but that this did not occur when the pathogenic HFE mutant C282Y was expressed. Furin, phosphoErk1/2 and phosphoSMAD1/5/8 were down-regulated also in transferrin receptor 2-null mice. Treatment of HepG2 cells with an inhibitor of furin activity caused a strong suppression of hepcidin mRNA, probably due to the inhibition of bone morphogenic protein maturation. CONCLUSIONS:The data indicate that transferrin receptor 2 and HFE are involved in holotransferrin-dependent signaling for the regulation of furin which involved Erk phosphorylation. Furin in turn may control hepcidin expression.
journal_name
Haematologicajournal_title
Haematologicaauthors
Poli M,Luscieti S,Gandini V,Maccarinelli F,Finazzi D,Silvestri L,Roetto A,Arosio Pdoi
10.3324/haematol.2010.027003subject
Has Abstractpub_date
2010-11-01 00:00:00pages
1832-40issue
11eissn
0390-6078issn
1592-8721pii
haematol.2010.027003journal_volume
95pub_type
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