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Abstract
Mutations in hemojuvelin (HJV) lead to the iron overload disorder juvenile hemochromatosis. HJV regulates iron metabolism by activating transcription of the iron regulatory peptide, hepcidin, through the bone morphogenetic protein (BMP) signaling pathway. HJV is proposed to act as a co-receptor for BMP ligands, but the exact mechanism by which it potentiates BMP signaling is not clear. To better understand the role of HJV in the regulation of iron metabolism, I analyzed its trafficking and processing. This work is the first to detail the trafficking of HJV and also increases our understanding of the proteolytic cleavage of HJV in response to iron demand.