Title |
Friedreich ataxia: metal dysmetabolism in dorsal root ganglia
|
---|---|
Published in |
Acta Neuropathologica Communications, June 2013
|
DOI | 10.1186/2051-5960-1-26 |
Pubmed ID | |
Authors |
Arnulf H Koeppen, Erik C Kuntzsch, Sarah T Bjork, R Liane Ramirez, Joseph E Mazurkiewicz, Paul J Feustel |
Abstract |
Friedreich ataxia (FA) causes distinctive lesions of dorsal root ganglia (DRG), including neuronal atrophy, satellite cell hyperplasia, and absorption of dying nerve cells into residual nodules. Two mechanisms may be involved: hypoplasia of DRG neurons from birth and superimposed iron (Fe)- and zinc (Zn)-mediated oxidative injury. This report presents a systematic analysis of DRG in 7 FA patients and 13 normal controls by X-ray fluorescence (XRF) of polyethylene glycol-embedded DRG; double-label confocal immunofluorescence microscopy of Zn- and Fe-related proteins; and immunohistochemistry of frataxin and the mitochondrial marker, ATP synthase F1 complex V β-polypeptide (ATP5B). |
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