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

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Ketogenic diet ameliorates axonal defects and promotes myelination in Pelizaeus-Merzbacher disease. / 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.

in: ACTA NEUROPATHOL, Jahrgang 138, Nr. 1, 07.2019, S. 147-161.

Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung

Harvard

Stumpf, SK, Berghoff, SA, Trevisiol, A, Spieth, L, Düking, T, Schneider, LV, Schlaphoff, L, Dreha-Kulaczewski, S, Bley, A, Burfeind, D, Kusch, K, Mitkovski, M, Ruhwedel, T, Guder, P, Röhse, H, Denecke, J, Gärtner, J, Möbius, W, Nave, K-A & Saher, G 2019, 'Ketogenic diet ameliorates axonal defects and promotes myelination in Pelizaeus-Merzbacher disease', ACTA NEUROPATHOL, Jg. 138, Nr. 1, S. 147-161. https://doi.org/10.1007/s00401-019-01985-2

APA

Stumpf, S. K., Berghoff, S. A., Trevisiol, A., Spieth, L., Düking, T., Schneider, L. V., Schlaphoff, L., Dreha-Kulaczewski, S., Bley, A., Burfeind, D., Kusch, K., Mitkovski, M., Ruhwedel, T., Guder, P., Röhse, H., Denecke, J., Gärtner, J., Möbius, W., Nave, K-A., & Saher, G. (2019). Ketogenic diet ameliorates axonal defects and promotes myelination in Pelizaeus-Merzbacher disease. ACTA NEUROPATHOL, 138(1), 147-161. https://doi.org/10.1007/s00401-019-01985-2

Vancouver

Stumpf SK, Berghoff SA, Trevisiol A, Spieth L, Düking T, Schneider LV et al. Ketogenic diet ameliorates axonal defects and promotes myelination in Pelizaeus-Merzbacher disease. ACTA NEUROPATHOL. 2019 Jul;138(1):147-161. https://doi.org/10.1007/s00401-019-01985-2

Bibtex

@article{5839c4493db3471a9400b41091b45423,
title = "Ketogenic diet ameliorates axonal defects and promotes myelination in Pelizaeus-Merzbacher disease",
abstract = "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.",
keywords = "Journal Article",
author = "Stumpf, {Sina K} and Berghoff, {Stefan A} and Andrea Trevisiol and Lena Spieth and Tim D{\"u}king and Schneider, {Lennart V} and Lennart Schlaphoff and Steffi Dreha-Kulaczewski and Annette Bley and Dinah Burfeind and Kathrin Kusch and Miso Mitkovski and Torben Ruhwedel and Philipp Guder and Heiko R{\"o}hse and Jonas Denecke and Jutta G{\"a}rtner and Wiebke M{\"o}bius and Klaus-Armin Nave and Gesine Saher",
year = "2019",
month = jul,
doi = "10.1007/s00401-019-01985-2",
language = "English",
volume = "138",
pages = "147--161",
journal = "ACTA NEUROPATHOL",
issn = "0001-6322",
publisher = "Springer",
number = "1",

}

RIS

TY - JOUR

T1 - Ketogenic diet ameliorates axonal defects and promotes myelination in Pelizaeus-Merzbacher disease

AU - Stumpf, Sina K

AU - Berghoff, Stefan A

AU - Trevisiol, Andrea

AU - Spieth, Lena

AU - Düking, Tim

AU - Schneider, Lennart V

AU - Schlaphoff, Lennart

AU - Dreha-Kulaczewski, Steffi

AU - Bley, Annette

AU - Burfeind, Dinah

AU - Kusch, Kathrin

AU - Mitkovski, Miso

AU - Ruhwedel, Torben

AU - Guder, Philipp

AU - Röhse, Heiko

AU - Denecke, Jonas

AU - Gärtner, Jutta

AU - Möbius, Wiebke

AU - Nave, Klaus-Armin

AU - Saher, Gesine

PY - 2019/7

Y1 - 2019/7

N2 - 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.

AB - 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.

KW - Journal Article

U2 - 10.1007/s00401-019-01985-2

DO - 10.1007/s00401-019-01985-2

M3 - SCORING: Journal article

C2 - 30919030

VL - 138

SP - 147

EP - 161

JO - ACTA NEUROPATHOL

JF - ACTA NEUROPATHOL

SN - 0001-6322

IS - 1

ER -