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Title
Peroxisomes in brain development and function
AuthorBerger, Johannes ; Dorninger, Fabian ; Forss-Petter, Sonja ; Kunze, Markus
Published in
Biochimica et Biophysica Acta - Molecular Cell Research, Amsterdam, 2016, Vol. 1863, Issue 5, page 934-955
PublishedAmsterdam : Elsevier, 2016
LanguageEnglish
Document typeJournal Article
Keywords (EN)lipid metabolism / plasmalogen / zellweger spectrum disorder / d-bifunctional protein deficiency / x-linked adrenoleukodystrophy / rhizomelic chondrodysplasia punctata / insulin-degrading enzyme / amino-acid oxidase / coa racemase deficiency / central-nervous-system / bifunctional protein-deficiency / adult refsum-disease / chain fatty-acids / ethanolamine plasmalogen deficiency
Project-/ReportnumberP 26112-B19
ISSN0167-4889
URNurn:nbn:at:at-ubmuw:3-1793 Persistent Identifier (URN)
DOI10.1016/j.bbamcr.2015.12.005 
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Abstract (English)

Peroxisomes contain numerous enzymatic activities that are important for mammalian physiology. Patients lacking either all peroxisomal functions or a single enzyme or transporter function typically developsevere neurological deficits, which originate from aberrant development of the brain, demyelination and loss of axonal integrity, neuroinfiammation or other neurodegenerative processes. Whilst correlating peroxisomal properties with a compilation of pathologies observed in human patients and mouse models lacking all or individual peroxisomal functions, we discuss the importance of peroxisomal metabolites and tissue- and cell type-specific contributions to the observed brain pathologies. This enables us to deconstruct the local and systemic contribution of individual metabolic pathways to specific brain functions. We also review the recently discovered variability of pathological symptoms in cases with unexpectedly mild presentation of peroxisome biogenesis disorders. Finally, we explore the emerging evidence linking peroxisomes to more common neurological disorders such as Alzheimer's disease, autism and amyotrophic lateral sclerosis. This article is part of a Special Issue entitled: Peroxisomes edited by Ralf Erdmann. (C) 2015 The Authors. Published by Elsevier B.V. This is an open access article under the CC BY license.

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CC-BY-License (4.0)Creative Commons Attribution 4.0 International License