title: A role for myelin-associated peroxisomes in maintaining paranodal loops and axonal integrity.
abstract: 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.
authors.affiliation: Department of Neurogenetics, Max Planck Institute of Experimental Medicine, Göttingen, Germany.
authors.forename: Celia M
authors.initials: CM
authors.lastname: Kassmann
authors.forename: Susanne
authors.initials: S
authors.lastname: Quintes
authors.forename: Jens
authors.initials: J
authors.lastname: Rietdorf
authors.forename: Wiebke
authors.initials: W
authors.lastname: Möbius
authors.forename: Michael Werner
authors.initials: MW
authors.lastname: Sereda
authors.forename: Tobias
authors.initials: T
authors.lastname: Nientiedt
authors.forename: Gesine
authors.initials: G
authors.lastname: Saher
authors.forename: Myriam
authors.initials: M
authors.lastname: Baes
authors.forename: Klaus-Armin
authors.initials: KA
authors.lastname: Nave
mesh_terms: D000818:Animals; D001369:Axons / Q000378:metabolism / Q000648:ultrastructure*; D051379:Mice; D008810:Mice, Inbred C57BL; D008822:Mice, Transgenic; D009186:Myelin Sheath / Q000378:metabolism*; D009413:Nerve Fibers, Myelinated / Q000378:metabolism / Q000648:ultrastructure*; D000074435:Peroxisome-Targeting Signal 1 Receptor; D020675:Peroxisomes / Q000378:metabolism*; D018160:Receptors, Cytoplasmic and Nuclear / Q000235:genetics / Q000378:metabolism; D012584:Sciatic Nerve / Q000378:metabolism / Q000648:ultrastructure
chemical_list: D000074435:Peroxisome-Targeting Signal 1 Receptor; C000614265:Pex5 protein, mouse; D018160:Receptors, Cytoplasmic and Nuclear