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    Pathological relationships involving iron and myelin may constitute a shared mechanism linking various rare and common brain diseases.

    247053_247053.pdf (1.300Mb)
    Access Status
    Open access
    Authors
    Heidari, M.
    Gerami, S.
    Bassett, B.
    Graham, Ross
    Chua, A.
    Aryal, R.
    House, M.
    Collingwood, J.
    Bettencourt, C.
    Houlden, H.
    Ryten, M.
    Olynyk, John
    Trinder, D.
    Johnstone, D.
    Milward, E.
    Date
    2016
    Type
    Journal Article
    
    Metadata
    Show full item record
    Citation
    Heidari, M. and Gerami, S. and Bassett, B. and Graham, R. and Chua, A. and Aryal, R. and House, M. et al. 2016. Pathological relationships involving iron and myelin may constitute a shared mechanism linking various rare and common brain diseases. Rare Disrease. 4 (1): pp. e1198458-1 - e1198458-11.
    Source Title
    Rare Dis
    DOI
    10.1080/21675511.2016.1198458
    School
    School of Biomedical Sciences
    Remarks

    This open access article is distributed under the Creative Commons license https://creativecommons.org/licenses/by-nc/3.0/

    URI
    http://hdl.handle.net/20.500.11937/3353
    Collection
    • Curtin Research Publications
    Abstract

    We previously demonstrated elevated brain iron levels in myelinated structures and associated cells in a hemochromatosis Hfe (-/-) xTfr2 (mut) mouse model. This was accompanied by altered expression of a group of myelin-related genes, including a suite of genes causatively linked to the rare disease family 'neurodegeneration with brain iron accumulation' (NBIA). Expanded data mining and ontological analyses have now identified additional myelin-related transcriptome changes in response to brain iron loading. Concordance between the mouse transcriptome changes and human myelin-related gene expression networks in normal and NBIA basal ganglia testifies to potential clinical relevance. These analyses implicate, among others, genes linked to various rare central hypomyelinating leukodystrophies and peripheral neuropathies including Pelizaeus-Merzbacher-like disease and Charcot-Marie-Tooth disease as well as genes linked to other rare neurological diseases such as Niemann-Pick disease. The findings may help understand interrelationships of iron and myelin in more common conditions such as hemochromatosis, multiple sclerosis and various psychiatric disorders.

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    • Brain iron accumulation affects myelin-related molecular systems implicated in a rare neurogenetic disease family with neuropsychiatric features
      Heidari, M.; Johnstone, D.; Bassett, B.; Graham, Ross; Chua, A.; House, M.; Collingwood, J.; Bettencourt, C.; Houlden, H.; Ryten, M.; Olynyk, John; Trinder, D.; Milward, E. (2016)
      The ‘neurodegeneration with brain iron accumulation’ (NBIA) disease family entails movement or cognitive impairment, often with psychiatric features. To understand how iron loading affects the brain, we studied mice with ...
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      Iron abnormalities within the brain are associated with several rare but severe neurodegenerative conditions. There is growing evidence that more common systemic iron loading disorders such as hemochromatosis can also ...
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