Article
Structure-function analysis of ferroportin defines the binding site and an alternative mechanism of action of hepcidin.
Blood - 22 Feb 2018
Aschemeyer Sharraya, Qiao Bo, Stefanova Deborah, Valore Erika V, Sek Albert C, Ruwe T Alex, Vieth Kyle R, Jung Grace, Casu Carla, Rivella Stefano, Jormakka Mika, Mackenzie Bryan, Ganz Tomas, Nemeth Elizabeta
Abstract excerpt
Nonclassical ferroportin disease (FD) is a form of hereditary hemochromatosis caused by mutations in the iron transporter ferroportin (Fpn), resulting in parenchymal iron overload. Fpn is regulated by the hormone hepcidin, which induces Fpn endocytosis and cellular iron retention. We characterized 11 clinically relevant and 5 nonclinical Fpn mutations using stably transfected, inducible isogenic cell lines. All...
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