FEBS letters, 2011-07, Vol.585 (14), p.2205-2211
2011
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Details
- Autor(en) / Beteiligte
- Titel
- A role for myelin-associated peroxisomes in maintaining paranodal loops and axonal integrity
- Ist Teil von
- FEBS letters, 2011-07, Vol.585 (14), p.2205-2211
- Ort / Verlag
- England: Elsevier B.V
- Erscheinungsjahr
- 2011
- Quelle
- Wiley-Blackwell Full Collection
- Beschreibungen/Notizen
- 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.
- Sprache
- Englisch
- Identifikatoren
- ISSN: 0014-5793eISSN: 1873-3468DOI: 10.1016/j.febslet.2011.05.032Titel-ID: cdi_proquest_miscellaneous_878031563
- Format
- –
- Schlagworte
- Adrenoleukodystrophy, Animals, Axons - metabolism, Axons - ultrastructure, Energy-metabolism, Mice, Mice, Inbred C57BL, Mice, Transgenic, Myelin, Myelin Sheath - metabolism, Nerve Fibers, Myelinated - metabolism, Nerve Fibers, Myelinated - ultrastructure, Peroxisome, Peroxisome-Targeting Signal 1 Receptor, Peroxisomes - metabolism, PEX5, Receptors, Cytoplasmic and Nuclear - genetics, Receptors, Cytoplasmic and Nuclear - metabolism, Sciatic Nerve - metabolism, Sciatic Nerve - ultrastructure