Peer-Reviewed Journal Details
Mandatory Fields
Bhatt, L,Horgan, CP,McCaffrey, MW;
2009
January
Biochemical and Biophysical Research Communications
Knockdown of beta 2-microglobulin perturbs the subcellular distribution of HFE and hepcidin
Validated
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Optional Fields
beta 2-microglobulin HFE Hepcidin Hereditary Haemochromatosis Late endosomes HEMOCHROMATOSIS PROTEIN HFE CELL-SURFACE EXPRESSION HEREDITARY HEMOCHROMATOSIS TRANSFERRIN RECEPTOR LATE ENDOSOMES IRON UPTAKE HLA-H BINDING BETA(2)-MICROGLOBULIN INTERNALIZATION
378
727
731
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. (C) 2008 Elsevier Inc. All rights reserved.
DOI 10.1016/j.bbrc.2008.11.118
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