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

Heidari, M; Gerami, SH; Bassett, B; Graham, RM; Chua, ACG; Aryal, R; House, MJ; ... Milward, EA; + view all (2016) Pathological relationships involving iron and myelin may constitute a shared mechanism linking various rare and common brain diseases. Rare Diseases , 4 (1) , Article e1198458. 10.1080/21675511.2016.1198458. Green open access

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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.

Type: Article
Title: Pathological relationships involving iron and myelin may constitute a shared mechanism linking various rare and common brain diseases
Location: United States
Open access status: An open access version is available from UCL Discovery
DOI: 10.1080/21675511.2016.1198458
Publisher version: http://doi.org/10.1080/21675511.2016.1198458
Language: English
Additional information: Copyright © 2016 Moones Heidari, Sam H. Gerami, Brianna Bassett, Ross M. Graham, Anita C.G. Chua, Ritambhara Aryal, Michael J. House, Joanna F. Collingwood, Conceic¸~ao Bettencourt, Henry Houlden, Mina Ryten, for the UK Brain Expression Consortium (UKBEC), John K. Olynyk, Debbie Trinder, Daniel M. Johnstone, and Elizabeth A. Milward. Published with license by Taylor & Francis. This is an Open Access article distributed under the terms of the Creative Commons Attribution-Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/), which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. The moral rights of the named author(s) have been asserted.
Keywords: NBIA, array, brain, hemochromatosis, iron, myelin, neurodegeneration, oligodendrocyte
UCL classification: UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Clinical and Movement Neurosciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Department of Neuromuscular Diseases
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Neurodegenerative Diseases
URI: http://discovery.ucl.ac.uk/id/eprint/1507532
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