Targeting myelin lipid metabolism as a potential therapeutic strategy in a model of CMT1A neuropathy

Charcot-Marie-Tooth disease 1A (CMT1A) is a peripheral demyelinating disease. Here, the authors demonstrate in a rodent model of CMT1A that Schwann cells have impairments in lipid biosynthesis, and that restoring lipids via diet can reverse the dysmyelinating phenotype in these animals.

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Main Authors: Fledrich, Robert (Author) , Abdelaal, T. (Author) , Rasch, L. (Author) , Bansal, V. (Author) , Schütza, V. (Author) , Brügger, Britta (Author) , Lüchtenborg, Christian (Author) , Prukop, T. (Author) , Stenzel, J. (Author) , Rahman, R. U. (Author) , Hermes, D. (Author) , Ewers, D. (Author) , Möbius, W. (Author) , Ruhwedel, T. (Author) , Katona, I. (Author) , Weis, J. (Author) , Klein, D. (Author) , Martini, R. (Author) , Brück, W. (Author) , Müller, W. C. (Author) , Bonn, S. (Author) , Bechmann, I. (Author) , Nave, K. A. (Author) , Stassart, Ruth Martha (Author) , Sereda, M. W. (Author)
Format: Article (Journal)
Language:English
Published: 02 August 2018
In: Nature Communications
Year: 2018, Volume: 9, Pages: 1-14
ISSN:2041-1723
DOI:10.1038/s41467-018-05420-0
Online Access:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1038/s41467-018-05420-0
Verlag, lizenzpflichtig, Volltext: https://www.nature.com/articles/s41467-018-05420-0
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Author Notes:R. Fledrich, T. Abdelaal, L. Rasch, V. Bansal, V. Schütza, B. Brügger, C. Lüchtenborg, T. Prukop, J. Stenzel, R.U. Rahman, D. Hermes, D. Ewers, W. Möbius, T. Ruhwedel, I. Katona, J. Weis, D. Klein, R. Martini, W. Brück, W.C. Müller, S. Bonn, I. Bechmann, K.A. Nave, R.M. Stassart and M.W. Sereda
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Summary:Charcot-Marie-Tooth disease 1A (CMT1A) is a peripheral demyelinating disease. Here, the authors demonstrate in a rodent model of CMT1A that Schwann cells have impairments in lipid biosynthesis, and that restoring lipids via diet can reverse the dysmyelinating phenotype in these animals.
Item Description:Gesehen am 22.04.2020
Physical Description:Online Resource
ISSN:2041-1723
DOI:10.1038/s41467-018-05420-0