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A role for myelin‐associated peroxisomes in maintaining paranodal loops and axonal integrity
Author(s) -
Kassmann Celia M.,
Quintes Susanne,
Rietdorf Jens,
Möbius Wiebke,
Sereda Michael Werner,
Nientiedt Tobias,
Saher Gesine,
Baes Myriam,
Nave Klaus-Armin
Publication year - 2011
Publication title -
febs letters
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.593
H-Index - 257
eISSN - 1873-3468
pISSN - 0014-5793
DOI - 10.1016/j.febslet.2011.05.032
Subject(s) - myelin , axon , microbiology and biotechnology , biogenesis , schwann cell , peripheral nervous system , peroxisome , neuroscience , biology , compartment (ship) , leukodystrophy , central nervous system , chemistry , pathology , biochemistry , medicine , receptor , oceanography , geology , gene , disease
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.