Ketogenic diet ameliorates axonal defects and promotes myelination in Pelizaeus-Merzbacher disease.
Axonal degeneration
Ketogenic diet
Mitochondria
Myelin
Pelizaeus–Merzbacher disease
Remyelination
Journal
Acta neuropathologica
ISSN: 1432-0533
Titre abrégé: Acta Neuropathol
Pays: Germany
ID NLM: 0412041
Informations de publication
Date de publication:
07 2019
07 2019
Historique:
received:
11
12
2018
accepted:
01
03
2019
revised:
25
02
2019
pubmed:
29
3
2019
medline:
22
7
2020
entrez:
29
3
2019
Statut:
ppublish
Résumé
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 not lead to a major advancement of hypomyelination, potentially because the intact blood-brain barrier precludes its entry into the CNS. We therefore turned to a PMD mouse model with preserved blood-brain barrier integrity and show that a high-fat/low-carbohydrate ketogenic diet restored oligodendrocyte integrity and increased CNS myelination. This dietary intervention also ameliorated axonal degeneration and normalized motor functions. Moreover, in a paradigm of adult remyelination, ketogenic diet facilitated repair and attenuated axon damage. We suggest that a therapy with lipids such as ketone bodies, that readily enter the brain, can circumvent the requirement of a disrupted blood-brain barrier in the treatment of myelin disease.
Identifiants
pubmed: 30919030
doi: 10.1007/s00401-019-01985-2
pii: 10.1007/s00401-019-01985-2
pmc: PMC6570703
doi:
Substances chimiques
Myelin Proteolipid Protein
0
Types de publication
Journal Article
Research Support, Non-U.S. Gov't
Langues
eng
Sous-ensembles de citation
IM
Pagination
147-161Commentaires et corrections
Type : ErratumIn
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