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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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Detalles Bibliográficos
Autores principales: 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: Online Artículo Texto
Lenguaje:English
Publicado: Springer Berlin Heidelberg 2019
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6570703/
https://www.ncbi.nlm.nih.gov/pubmed/30919030
http://dx.doi.org/10.1007/s00401-019-01985-2