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Reply to “Iron homeostasis in the brain: complete iron regulatory protein 2 deficiency without symptomatic neurodegeneration in the mouse”

Nature Genetics volume 38, pages 969–970 (2006)Cite this article

Ghosh et al. reply:

We previously reported that targeted deletion of Ireb2, the gene encoding iron regulatory protein 2 (IRP2), caused misregulation of iron metabolism in mice, including overexpression of ferritin and decreased expression of the transferrin receptor in multiple tissues1, whereas targeted deletion of Aco1, the gene encoding iron regulatory protein 1 (IRP1), a highly homologous gene, was associated with a minimal perturbation of iron metabolism in mice2,3. The Ireb2−/− mice developed adult-onset, slowly progressive neurodegeneration1 and iron-insufficiency anemia associated with erythropoietic protoporphyria4, and both the neurodegeneration and anemia were more pronounced in mice that lacked both copies of Ireb2 and one copy of Aco1 (Aco1+/− Ireb2−/−)5.

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Figure 1: Deletion of one allele of Aco1 exacerbates neurodegeneration and diminishes life span of Ireb2−/− mice.

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Authors and Affiliations

  1. Cell Biology and Metabolism Branch, National Institute of Child Health and Human Development, US National Institutes of Health, Bethesda, Maryland, USA

    Manik C Ghosh, Hayden Ollivierre-Wilson, Sharon Cooperman & Tracey A Rouault

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  1. Manik C Ghosh
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  2. Hayden Ollivierre-Wilson
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  3. Sharon Cooperman
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  4. Tracey A Rouault
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Ghosh, M., Ollivierre-Wilson, H., Cooperman, S. et al. Reply to “Iron homeostasis in the brain: complete iron regulatory protein 2 deficiency without symptomatic neurodegeneration in the mouse”. Nat Genet 38, 969–970 (2006). https://doi.org/10.1038/ng0906-969

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