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  • Mitochondrial changes and oxidative stress in a mouse model of Zellweger syndrome neuropathogenesis

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    Author(s)
    Rahim, Rani Sadia
    Chen, Mo
    Nourse, C Cathrin
    Meedeniya, Adrian CB
    Crane, Denis I
    Griffith University Author(s)
    Crane, Denis I.
    Chen, Mo
    Year published
    2016
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    Abstract
    Zellweger syndrome (ZS) is a peroxisome biogenesis disorder that involves significant neuropathology, the molecular basis of which is still poorly understood. Using a mouse model of ZS with brain-restricted deficiency of the peroxisome biogenesis protein PEX13, we demonstrated an expanded and morphologically modified brain mitochondrial population. Cultured fibroblasts from PEX13-deficient mouse embryo displayed similar changes, as well as increased levels of mitochondrial superoxide and membrane depolarization; this phenotype was rescued by antioxidant treatment. Significant oxidative damage to neurons in brain was indicated ...
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    Zellweger syndrome (ZS) is a peroxisome biogenesis disorder that involves significant neuropathology, the molecular basis of which is still poorly understood. Using a mouse model of ZS with brain-restricted deficiency of the peroxisome biogenesis protein PEX13, we demonstrated an expanded and morphologically modified brain mitochondrial population. Cultured fibroblasts from PEX13-deficient mouse embryo displayed similar changes, as well as increased levels of mitochondrial superoxide and membrane depolarization; this phenotype was rescued by antioxidant treatment. Significant oxidative damage to neurons in brain was indicated by products of lipid and DNA oxidation. Similar overall changes were observed for glial cells. In toto, these findings suggest that mitochondrial oxidative stress and aberrant mitochondrial dynamics are associated with the neuropathology arising from PEX13 deficiency.
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    Journal Title
    Neuroscience
    Volume
    334
    DOI
    https://doi.org/10.1016/j.neuroscience.2016.08.001
    Copyright Statement
    © 2016 International Brain Research Organization. Published by Elsevier Ltd. Licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International Licence (http://creativecommons.org/licenses/by-nc-nd/4.0/) which permits unrestricted, non-commercial use, distribution and reproduction in any medium, providing that the work is properly cited.
    Subject
    Cell neurochemistry
    Neurosciences
    Psychology
    Cognitive and computational psychology
    Publication URI
    http://hdl.handle.net/10072/100002
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    • Journal articles

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