Journal
FEBS LETTERS
Volume 585, Issue 14, Pages 2205-2211Publisher
ELSEVIER SCIENCE BV
DOI: 10.1016/j.febslet.2011.05.032
Keywords
Myelin; Peroxisome; PEX5; Adrenoleukodystrophy; Energy-metabolism
Funding
- EU
- DFG [SFB/TR43]
- Oliver's Army
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Demyelinating diseases of the nervous system cause axon loss but the underlying mechanisms are not well understood. Here we show by confocal and electron microscopy that in myelin-forming glia peroxisomes are associated with myelin membranes. When peroxisome biogenesis is experimentally perturbed in Pex5 conditional mouse mutants, myelination by Schwann cells appears initially normal. However, in nerves of older mice paranodal loops become physically unstable and develop swellings filled with vesicles and electron-dense material. This novel model of a demyelinating neuropathy demonstrates that peroxisomes serve an important function in the peripheral myelin compartment, required for long-term axonal integrity. (C) 2011 Federation of European Biochemical Societies. Published by Elsevier B.V. All rights reserved.
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