Article ID | Journal | Published Year | Pages | File Type |
---|---|---|---|---|
1934970 | Biochemical and Biophysical Research Communications | 2009 | 5 Pages |
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 (β2M). Here, we report that knockdown of β2M 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 β2M by RNAi perturbs hepcidin localisation to late endosomes. In summary, our data suggest that β2M is essential for the correct subcellular distribution of both HFE and hepcidin, two proteins, which are critical for iron homeostasis.
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Authors
Lavinia Bhatt, Conor P. Horgan, Mary W. McCaffrey,