Journal article
Pathological relationships involving iron and myelin may constitute a shared mechanism linking various rare and common brain diseases
Rare Diseases, Vol.4(1), e1198458
2016
Abstract
We previously demonstrated elevated brain iron levels in myelinated structures and associated cells in a hemochromatosis Hfe−/−xTfr2mut 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.
Details
- Title
- Pathological relationships involving iron and myelin may constitute a shared mechanism linking various rare and common brain diseases
- Authors/Creators
- M. Heidari (Author/Creator) - University of Newcastle AustraliaS.H. Gerami (Author/Creator) - University of Newcastle AustraliaB. Bassett (Author/Creator) - University of Newcastle AustraliaR.M. Graham (Author/Creator) - Curtin UniversityA.C.G. Chua (Author/Creator) - Fiona Stanley HospitalR. Aryal (Author/Creator) - University of Newcastle AustraliaM.J. House (Author/Creator) - The University of Western AustraliaJ.F. Collingwood (Author/Creator) - University of WarwickC. Bettencourt (Author/Creator) - UCL Institute of NeurologyH. Houlden (Author/Creator) - UCL Institute of NeurologyM. Ryten (Author/Creator) - UCL Institute of NeurologyJ.K. Olynyk (Author/Creator) - Curtin UniversityD. Trinder (Author/Creator) - Fiona Stanley HospitalD.M. Johnstone (Author/Creator) - The University of SydneyE.A. Milward (Author/Creator) - University of Newcastle Australia
- Publication Details
- Rare Diseases, Vol.4(1), e1198458
- Publisher
- Taylor & Francis Group, LLC
- Identifiers
- 991005542237907891
- Copyright
- © 2016 The Author(s).
- Murdoch Affiliation
- Murdoch University
- Language
- English
- Resource Type
- Journal article
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