Dept. of Cell and Developmental Biology
Oregon Health & Science University
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. HJV traffics to the cell surface while avoiding modification by glycosylases and proteases that reside in the Golgi. After reaching the cell surface, HJV undergoes retrograde trafficking to the Golgi, where it is further modified and cleaved by furin. HJV is cleaved by the furin family of proprotein convertases, which generates a soluble form of HJV that antagonizes BMP signaling. Reaching the cell surface prior to cleavage and secretion may allow HJV to participate in BMP signaling at th
School of Medicine
Maxson, Julia Elizabeth, "Processing and trafficking of the iron regulatory protein, hemojuvelin" (2011). Scholar Archive. 667.