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Nutr Rev. 2009 May;67(5):284-8. doi: 10.1111/j.1753-4887.2009.00200.x.

Into the matrix: regulation of the iron regulatory hormone hepcidin by matriptase-2.

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Food Science and Human Nutrition Department, University of Florida, Gainesville, Florida 32611, USA.


Matriptase-2 is a recently identified membrane-bound, cell-surface serine protease expressed primarily in liver. Mutations in matriptase-2 in mice and humans cause iron-deficiency anemia that responds poorly to iron therapy. The poor response results from an inability to decrease hepcidin production during iron deficiency. Cell culture studies reveal that matriptase-2 inhibits hepcidin induction by cleaving membrane hemojuvelin, a potent activator of hepcidin transcription. As a novel suppressor of hepcidin expression, matriptase-2 emerges as a possible candidate for therapeutic interventions aimed at treating disorders of iron metabolism.

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