Journal article
Iron Accumulates in Huntington's Disease Neurons: Protection by Deferoxamine
J Chen, E Marks, B Lai, Z Zhang, JA Duce, LQ Lam, I Volitakis, AI Bush, S Hersch, JH Fox
Plos One | Published : 2013
Abstract
Huntington's disease (HD) is a progressive neurodegenerative disorder caused by a polyglutamine-encoding CAG expansion in the huntingtin gene. Iron accumulates in the brains of HD patients and mouse disease models. However, the cellular and subcellular sites of iron accumulation, as well as significance to disease progression are not well understood. We used independent approaches to investigate the location of brain iron accumulation. In R6/2 HD mouse brain, synchotron x-ray fluorescence analysis revealed iron accumulation as discrete puncta in the perinuclear cytoplasm of striatal neurons. Further, perfusion Turnbull's staining for ferrous iron (II) combined with transmission electron micr..
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Grants
Awarded by National Center for Research Resources
Funding Acknowledgements
This work was supported by grants from the National Institutes of Health and by the Huntington's Disease Society of America, the National Center for Research Resources (5P30RR032128-02), the National Institute of General Medical Sciences (8 P30GM103398-02) and National Institute of Neurological Disorders and Stroke (RO1NS079450) from the National Institutes of Health. Use of the Advanced Photon Source at Argonne National Laboratory was supported by the U. S. Department of Energy, Office of Science, Office of Basic Energy Sciences, under Contract No. DE-AC02-06CH11357. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.