Evidence map›Paper›PMID 30919030›Full record

ArticleActa neuropathologica2019

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

Sina K Stumpf, Stefan A Berghoff, Andrea Trevisiol, Lena Spieth, Tim Düking, Lennart V Schneider, Lennart Schlaphoff, Steffi Dreha-Kulaczewski, Annette Bley, Dinah Burfeind and 10 more

Erratum issuedOpen access · hybridAbstract read
In one paragraph

Article in Acta neuropathologica, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 41 papers.

0numbers the graph read from it
0cells of the map it votes in
41citing papers in PubMed
6.1field-weighted citation impact, top 3% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

41 citing papers in PubMed, 79 citations in OpenAlex.

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4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

20 authors at 4 institutions in 1 country.

Sina K StumpfDepartment of Neurogenetics, Max-Planck-Institute of Experimental Medicine, Hermann-Rein-Str. 3, 37075, Göttingen, Germany.
Stefan A BerghoffDepartment of Neurogenetics, Max-Planck-Institute of Experimental Medicine, Hermann-Rein-Str. 3, 37075, Göttingen, Germany.
Andrea TrevisiolDepartment of Neurogenetics, Max-Planck-Institute of Experimental Medicine, Hermann-Rein-Str. 3, 37075, Göttingen, Germany.
Lena SpiethDepartment of Neurogenetics, Max-Planck-Institute of Experimental Medicine, Hermann-Rein-Str. 3, 37075, Göttingen, Germany.
Tim DükingDepartment of Neurogenetics, Max-Planck-Institute of Experimental Medicine, Hermann-Rein-Str. 3, 37075, Göttingen, Germany.
Lennart V SchneiderDepartment of Neurogenetics, Max-Planck-Institute of Experimental Medicine, Hermann-Rein-Str. 3, 37075, Göttingen, Germany.
Lennart SchlaphoffDepartment of Neurogenetics, Max-Planck-Institute of Experimental Medicine, Hermann-Rein-Str. 3, 37075, Göttingen, Germany.
Steffi Dreha-KulaczewskiDivision of Pediatric Neurology, Department of Pediatrics and Adolescent Medicine, University Medical Center, 37075, Göttingen, Germany.
Annette BleyUniversity Children's Hospital, University Medical Center Hamburg-Eppendorf, 20246, Hamburg, Germany.
Dinah BurfeindDepartment of Neurogenetics, Max-Planck-Institute of Experimental Medicine, Hermann-Rein-Str. 3, 37075, Göttingen, Germany.
Kathrin KuschDepartment of Neurogenetics, Max-Planck-Institute of Experimental Medicine, Hermann-Rein-Str. 3, 37075, Göttingen, Germany.
Miso MitkovskiLight Microscopy Facility, Max-Planck-Institute of Experimental Medicine, 37075, Göttingen, Germany.
Torben RuhwedelDepartment of Neurogenetics, Max-Planck-Institute of Experimental Medicine, Hermann-Rein-Str. 3, 37075, Göttingen, Germany.
Philipp GuderUniversity Children's Hospital, University Medical Center Hamburg-Eppendorf, 20246, Hamburg, Germany.
Heiko RöhseLight Microscopy Facility, Max-Planck-Institute of Experimental Medicine, 37075, Göttingen, Germany.
Jonas DeneckeUniversity Children's Hospital, University Medical Center Hamburg-Eppendorf, 20246, Hamburg, Germany.
Jutta GärtnerDivision of Pediatric Neurology, Department of Pediatrics and Adolescent Medicine, University Medical Center, 37075, Göttingen, Germany.
Wiebke MöbiusDepartment of Neurogenetics, Max-Planck-Institute of Experimental Medicine, Hermann-Rein-Str. 3, 37075, Göttingen, Germany.
Klaus-Armin NaveDepartment of Neurogenetics, Max-Planck-Institute of Experimental Medicine, Hermann-Rein-Str. 3, 37075, Göttingen, Germany.
Gesine SaherDepartment of Neurogenetics, Max-Planck-Institute of Experimental Medicine, Hermann-Rein-Str. 3, 37075, Göttingen, Germany. saher@em.mpg.de.ORCID 0000-0003-3507-9604
Max Planck Institute of Experimental Medicine · DEUniversität Hamburg · DENanoscale Microscopy and Molecular Physiology of the Brain Cluster of Excellence 171 — DFG Research Center 103 · DEUniversity of Göttingen · DE

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

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.

Indexed as

AnimalsDemyelinating DiseasesDiet, KetogenicDisease Models, AnimalMiceMyelin Proteolipid ProteinOligodendrogliaOrganogenesisPelizaeus-Merzbacher DiseaseMyelin Proteolipid ProteinAxonal degenerationKetogenic dietMitochondriaMyelinPelizaeus–Merzbacher diseaseRemyelination

Identifiers

PMID30919030
PMCPMC6570703
OpenAlexW2924166170

What OpenQuestion holds

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LicenceCC BY
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.