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.

Bibliographic Details
Main Authors: 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, M. W. Sereda
Format: Article
Language:English
Published: Nature Publishing Group 2018-08-01
Series:Nature Communications
Online Access:https://doi.org/10.1038/s41467-018-05420-0
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spelling doaj-8b8e4e83c023447f9c6cbe01a5ec8a8b2021-05-11T09:48:20ZengNature Publishing GroupNature Communications2041-17232018-08-019111410.1038/s41467-018-05420-0Targeting myelin lipid metabolism as a potential therapeutic strategy in a model of CMT1A neuropathyR. Fledrich0T. Abdelaal1L. Rasch2V. Bansal3V. Schütza4B. Brügger5C. Lüchtenborg6T. Prukop7J. Stenzel8R. U. Rahman9D. Hermes10D. Ewers11W. Möbius12T. Ruhwedel13I. Katona14J. Weis15D. Klein16R. Martini17W. Brück18W. C. Müller19S. Bonn20I. Bechmann21K. A. Nave22R. M. Stassart23M. W. Sereda24Department of Neurogenetics, Max-Planck-Institute of Experimental MedicineDepartment of Neurogenetics, Max-Planck-Institute of Experimental MedicineDepartment of Neurogenetics, Max-Planck-Institute of Experimental MedicineCenter for Molecular Neurobiology, Institute of Medical Systems Biology, University Medical Center Hamburg-EppendorfDepartment of Neurogenetics, Max-Planck-Institute of Experimental MedicineHeidelberg University Biochemistry Center (BZH)Heidelberg University Biochemistry Center (BZH)Department of Neurogenetics, Max-Planck-Institute of Experimental MedicineDepartment of Neurogenetics, Max-Planck-Institute of Experimental MedicineCenter for Molecular Neurobiology, Institute of Medical Systems Biology, University Medical Center Hamburg-EppendorfDepartment of Neurogenetics, Max-Planck-Institute of Experimental MedicineDepartment of Neurogenetics, Max-Planck-Institute of Experimental MedicineDepartment of Neurogenetics, Max-Planck-Institute of Experimental MedicineDepartment of Neurogenetics, Max-Planck-Institute of Experimental MedicineInstitute of Neuropathology, University Hospital AachenInstitute of Neuropathology, University Hospital AachenDepartment of Neurology, Section of Developmental Neurobiology, University Hospital WuerzburgDepartment of Neurology, Section of Developmental Neurobiology, University Hospital WuerzburgInstitute of Neuropathology, University Medical Center GöttingenDepartment of Neuropathology, University Hospital LeipzigCenter for Molecular Neurobiology, Institute of Medical Systems Biology, University Medical Center Hamburg-EppendorfInstitute of Anatomy, University of LeipzigDepartment of Neurogenetics, Max-Planck-Institute of Experimental MedicineDepartment of Neurogenetics, Max-Planck-Institute of Experimental MedicineDepartment of Neurogenetics, Max-Planck-Institute of Experimental MedicineCharcot–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.https://doi.org/10.1038/s41467-018-05420-0
collection DOAJ
language English
format Article
sources DOAJ
author 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
M. W. Sereda
spellingShingle 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
M. W. Sereda
Targeting myelin lipid metabolism as a potential therapeutic strategy in a model of CMT1A neuropathy
Nature Communications
author_facet 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
M. W. Sereda
author_sort R. Fledrich
title Targeting myelin lipid metabolism as a potential therapeutic strategy in a model of CMT1A neuropathy
title_short Targeting myelin lipid metabolism as a potential therapeutic strategy in a model of CMT1A neuropathy
title_full Targeting myelin lipid metabolism as a potential therapeutic strategy in a model of CMT1A neuropathy
title_fullStr Targeting myelin lipid metabolism as a potential therapeutic strategy in a model of CMT1A neuropathy
title_full_unstemmed Targeting myelin lipid metabolism as a potential therapeutic strategy in a model of CMT1A neuropathy
title_sort targeting myelin lipid metabolism as a potential therapeutic strategy in a model of cmt1a neuropathy
publisher Nature Publishing Group
series Nature Communications
issn 2041-1723
publishDate 2018-08-01
description 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.
url https://doi.org/10.1038/s41467-018-05420-0
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