부산대학교 도서관

To link to full-text access for this article, visit this link: http://dx.doi.org/10.1016/j.bbrc.2008.11.118 Byline: Lavinia Bhatt, Conor P. Horgan, Mary W. McCaffrey Keywords: [beta]2-microglobulin; HFE; Hepcidin; Hereditary Haemochromatosis; Late endosomes Abstract: Hereditary Haemochromatosis is an iron overload disorder associated with mutations in the HFE gene, and to a lesser degree, the gene encoding its chaperone protein beta-2 microglobulin ([beta]2M). Here, we report that knockdown of [beta]2M by RNAi restricts HFE distribution to the endoplasmic reticulum (ER). Additionally, we demonstrate that hepcidin, an iron homeostasis-associated protein, localises predominantly to LBPA-positive late endosomes. Interestingly, we show that knockdown of [beta]2M by RNAi perturbs hepcidin localisation to late endosomes. In summary, our data suggest that [beta]2M is essential for the correct subcellular distribution of both HFE and hepcidin, two proteins, which are critical for iron homeostasis. Author Affiliation: Molecular Cell Biology Laboratory, Department of Biochemistry, Biosciences Institute, University College Cork, Cork, Ireland Article History: Received 4 November 2008