Ferroportin (Fpn) disease mouse model


CU investigators have created the first mouse model for ferroportin (Fpn) disease, also known as hemochromatosis type IV.  Using the ffe mouse line, investigators have induced a missense mutation in Fpn (H32R) that affects the localization of the iron transporter and its iron export activity.  These mice present with all of the features of human Fpn disease- accumulation of iron in Kupffer cells, high serum ferritin, and low transferrin saturation.  This creates an excellent model to study Fpn disease and the pathophysiological consequences of Kupffer cell iron loading.


Zohn IE, De domenico I, Pollock A, et al. The flatiron mutation in mouse ferroportin acts as a dominant negative to cause ferroportin disease. Blood. 2007;109(10):4174-80.

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Aaron Troemner
University of Colorado
Lee Niswander
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