Iron Homeostasis in the CNS: An Overview of the Pathological Consequences of Iron Metabolism Disruption |
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Authors: | Christina A Porras Tracey A Rouault |
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Affiliation: | Eunice Kennedy Shriver National Institute of Child Health and Development, Bethesda, MD 20892, USA; |
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Abstract: | Iron homeostasis disruption has increasingly been implicated in various neurological disorders. In this review, we present an overview of our current understanding of iron metabolism in the central nervous system. We examine the consequences of both iron accumulation and deficiency in various disease contexts including neurodegenerative, neurodevelopmental, and neuropsychological disorders. The history of animal models of iron metabolism misregulation is also discussed followed by a comparison of three patients with a newly discovered neurodegenerative disorder caused by mutations in iron regulatory protein 2. |
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Keywords: | iron metabolism iron homeostasis iron regulatory proteins neurodegeneration iron accumulation iron deficiency |
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