Nature Communications (Aug 2018)
Targeting myelin lipid metabolism as a potential therapeutic strategy in a model of CMT1A neuropathy
- 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
Affiliations
- R. Fledrich
- Department of Neurogenetics, Max-Planck-Institute of Experimental Medicine
- T. Abdelaal
- Department of Neurogenetics, Max-Planck-Institute of Experimental Medicine
- L. Rasch
- Department of Neurogenetics, Max-Planck-Institute of Experimental Medicine
- V. Bansal
- Center for Molecular Neurobiology, Institute of Medical Systems Biology, University Medical Center Hamburg-Eppendorf
- V. Schütza
- Department of Neurogenetics, Max-Planck-Institute of Experimental Medicine
- B. Brügger
- Heidelberg University Biochemistry Center (BZH)
- C. Lüchtenborg
- Heidelberg University Biochemistry Center (BZH)
- T. Prukop
- Department of Neurogenetics, Max-Planck-Institute of Experimental Medicine
- J. Stenzel
- Department of Neurogenetics, Max-Planck-Institute of Experimental Medicine
- R. U. Rahman
- Center for Molecular Neurobiology, Institute of Medical Systems Biology, University Medical Center Hamburg-Eppendorf
- D. Hermes
- Department of Neurogenetics, Max-Planck-Institute of Experimental Medicine
- D. Ewers
- Department of Neurogenetics, Max-Planck-Institute of Experimental Medicine
- W. Möbius
- Department of Neurogenetics, Max-Planck-Institute of Experimental Medicine
- T. Ruhwedel
- Department of Neurogenetics, Max-Planck-Institute of Experimental Medicine
- I. Katona
- Institute of Neuropathology, University Hospital Aachen
- J. Weis
- Institute of Neuropathology, University Hospital Aachen
- D. Klein
- Department of Neurology, Section of Developmental Neurobiology, University Hospital Wuerzburg
- R. Martini
- Department of Neurology, Section of Developmental Neurobiology, University Hospital Wuerzburg
- W. Brück
- Institute of Neuropathology, University Medical Center Göttingen
- W. C. Müller
- Department of Neuropathology, University Hospital Leipzig
- S. Bonn
- Center for Molecular Neurobiology, Institute of Medical Systems Biology, University Medical Center Hamburg-Eppendorf
- I. Bechmann
- Institute of Anatomy, University of Leipzig
- K. A. Nave
- Department of Neurogenetics, Max-Planck-Institute of Experimental Medicine
- R. M. Stassart
- Department of Neurogenetics, Max-Planck-Institute of Experimental Medicine
- M. W. Sereda
- Department of Neurogenetics, Max-Planck-Institute of Experimental Medicine
- DOI
- https://doi.org/10.1038/s41467-018-05420-0
- Journal volume & issue
-
Vol. 9,
no. 1
pp. 1 – 14
Abstract
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.
