Ketogenic diet ameliorates axonal defects and promotes myelination in Pelizaeus–Merzbacher disease

Pelizaeus–Merzbacher disease (PMD) is an untreatable and fatal leukodystrophy. In a model of PMD with perturbed blood–brain barrier integrity, cholesterol supplementation promotes myelin membrane growth. Here, we show that in contrast to the mouse model, dietary cholesterol in two PMD patients did n...

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Detalhes bibliográficos
Publicado no:Acta Neuropathol
Main Authors: Stumpf, Sina K., Berghoff, Stefan A., Trevisiol, Andrea, Spieth, Lena, Düking, Tim, Schneider, Lennart V., Schlaphoff, Lennart, Dreha-Kulaczewski, Steffi, Bley, Annette, Burfeind, Dinah, Kusch, Kathrin, Mitkovski, Miso, Ruhwedel, Torben, Guder, Philipp, Röhse, Heiko, Denecke, Jonas, Gärtner, Jutta, Möbius, Wiebke, Nave, Klaus-Armin, Saher, Gesine
Formato: Artigo
Idioma:Inglês
Publicado em: Springer Berlin Heidelberg 2019
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Original Paper
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC6570703/
https://ncbi.nlm.nih.gov/pubmed/30919030
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1007/s00401-019-01985-2
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