Correction to: 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, Mišo; Ruhwedel, Torben; Guder, Philipp; Röhse, Heiko; Denecke, Jonas; Gärtner, Jutta; Möbius, Wiebke; Nave, Klaus‐Armin; Saher, Gesine

doi:10.1007/s00401-019-02064-2

Cited by 6 publications

(4 citation statements)

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“…Brain hemispheres were embedded in paraffin, sectioned coronally, and labeled chromogenic for CD3, GFAP, APP, and MAC3 and with fluorescent antibodies for CAII, Olig2, and PDGFRa as essentially described in refs. 78 , 79 . TUNEL staining of paraffin fimbria sections for quantification of apoptotic cells was performed according to the manufacturer’s protocols (DeadEnd™ Colorimetric TUNEL System, Promega).…”

Section: Methodsmentioning

confidence: 99%

White matter integrity in mice requires continuous myelin synthesis at the inner tongue

et al. 2022

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Myelin, the electrically insulating sheath on axons, undergoes dynamic changes over time. However, it is composed of proteins with long lifetimes. This raises the question how such a stable structure is renewed. Here, we study the integrity of myelinated tracts after experimentally preventing the formation of new myelin in the CNS of adult mice, using an inducible Mbp null allele. Oligodendrocytes survive recombination, continue to express myelin genes, but they fail to maintain compacted myelin sheaths. Using 3D electron microscopy and mass spectrometry imaging we visualize myelin-like membranes failing to incorporate adaxonally, most prominently at juxta-paranodes. Myelinoid body formation indicates degradation of existing myelin at the abaxonal side and the inner tongue of the sheath. Thinning of compact myelin and shortening of internodes result in the loss of about 50% of myelin and axonal pathology within 20 weeks post recombination. In summary, our data suggest that functional axon-myelin units require the continuous incorporation of new myelin membranes.

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Section: Methodsmentioning

confidence: 99%

White matter integrity in mice requires continuous myelin synthesis at the inner tongue

et al. 2022

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“…This study explored the relevant mechanism of short-term KD treatment in SCI rats [ 37 ]. Additionally, studies have found that HDLc showed small increases after ketogenic phases, but there was no significant change over 12 months [ 38 ].…”

Section: Discussionmentioning

confidence: 99%

Ketogenic diet-mediated steroid metabolism reprogramming improves the immune microenvironment and myelin growth in spinal cord injury rats according to gene and co-expression network analyses

Zeng

Huang

et al. 2021

Aging

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The ketogenic diet has been widely used in the treatment of various nervous system and metabolic-related diseases. Our previous research found that a ketogenic diet exerts a protective effect and promotes functional recovery after spinal cord injury. However, the mechanism of action is still unclear. In this study, different dietary feeding methods were used, and myelin expression and gene level changes were detected among different groups. We established 15 RNA-seq cDNA libraries from among 4 different groups. First, KEGG pathway enrichment of upregulated differentially expressed genes and gene set enrichment analysis of the ketogenic diet and normal diet groups indicated that a ketogenic diet significantly improved the steroid anabolic pathway in rats with spinal cord injury. Through cluster analysis, protein-protein interaction analysis and visualization of iPath metabolic pathways, it was determined that Sqle, Sc5d, Cyp51, Dhcr24, Msmo1, Hsd17b7, and Fdft1 expression changed significantly. Second, through weighted gene co-expression network analysis showed that rats fed a ketogenic diet showed a significant reduction in the expression of genes involved in immune-related pathways, including those associated with immunity and infectious diseases. A ketogenic diet may improve the immune microenvironment and myelin growth in rats with spinal cord injury through reprogramming of steroid metabolism.

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“…Dietary cholesterol supplementation does not cure PMD but rather helps relieve the oligodendrocytes from the cytoplasmic accumulation of PLP1 [ 133 ]. The ketogenic diet, which is high in fat and low in carbohydrates, helps to restore the oligodendrocytes, thus improving myelination in +PlpTg 72/72 mice due to the ketone bodies that can cross the blood–brain barrier and that enhance lipid synthesis [ 134 ].…”

Section: Therapiesmentioning

confidence: 99%

Mutation of Proteolipid Protein 1 Gene: From Severe Hypomyelinating Leukodystrophy to Inherited Spastic Paraplegia

Khalaf

Mattern²,

Bégou

et al. 2022

Biomedicines

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Pelizaeus–Merzbacher Disease (PMD) is an inherited leukodystrophy affecting the central nervous system (CNS)—a rare disorder that especially concerns males. Its estimated prevalence is 1.45–1.9 per 100,000 individuals in the general population. Patients affected by PMD exhibit a drastic reduction or absence of myelin sheaths in the white matter areas of the CNS. The Proteolipid Protein 1 (PLP1) gene encodes a transmembrane proteolipid protein. PLP1 is the major protein of myelin, and it plays a key role in the compaction, stabilization, and maintenance of myelin sheaths. Its function is predominant in oligodendrocyte development and axonal survival. Mutations in the PLP1 gene cause the development of a wide continuum spectrum of leukopathies from the most severe form of PMD for whom patients exhibit severe CNS hypomyelination to the relatively mild late-onset type 2 spastic paraplegia, leading to the concept of PLP1-related disorders. The genetic diversity and the biochemical complexity, along with other aspects of PMD, are discussed to reveal the obstacles that hinder the development of treatments. This review aims to provide a clinical and mechanistic overview of this spectrum of rare diseases.

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

Abstract: The original article was published with an erroneously duplicated image in Fig. 2a. The corrected Fig. 2a is given in the next page.

Cited by 6 publications

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White matter integrity in mice requires continuous myelin synthesis at the inner tongue

White matter integrity in mice requires continuous myelin synthesis at the inner tongue

Ketogenic diet-mediated steroid metabolism reprogramming improves the immune microenvironment and myelin growth in spinal cord injury rats according to gene and co-expression network analyses

Mutation of Proteolipid Protein 1 Gene: From Severe Hypomyelinating Leukodystrophy to Inherited Spastic Paraplegia

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