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Cited by 91Sphingolipid Metabolism and SignalingLysosomal Storage Disorders ResearchMultiple Sclerosis Research Studies

The role of Sphingolipids in myelination and myelin stability and their involvement in childhood and adult demyelinating disorders

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Giussani, Paola, Prinetti, Alessandro, Tringali, Cristina·2021·Journal of Neurochemistry
DOI 10.1111/jnc.15133
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Abstract

Multiple sclerosis (MS) represents the most common demyelinating disease affecting the central nervous system (CNS) in adults as well as in children. Furthermore, in children, in addition to acquired diseases such as MS, genetically inherited diseases significantly contribute to the incidence of demyelinating disorders. Some genetic defects lead to sphingolipid alterations that are able to elicit neurological symptoms. Sphingolipids are essential for brain development, and their aberrant functionality may thus contribute to demyelinating diseases such as MS. In particular, sphingolipidoses caused by deficits of sphingolipid‐metabolizing enzymes, are often associated with demyelination. Sphingolipids are not only structural molecules but also bioactive molecules involved in the regulation of cellular events such as development of the nervous system, myelination and maintenance of myelin stability. Changes in the sphingolipid metabolism deeply affect plasma membrane organization. Thus, changes in myelin sphingolipid composition might crucially contribute to the phenotype of diseases characterized by demyelinalization. Here, we review key features of several sphingolipids such as ceramide/dihydroceramide, sphingosine/dihydrosphingosine, glucosylceramide and, galactosylceramide which act in myelin formation during rat brain development and in human brain demyelination during the pathogenesis of MS, suggesting that this knowledge could be useful in identifying targets for possible therapies. image

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  • Pathways upregulated by OL2 were involved in synthesis of several lipid subtypes, including glycosphingolipids and sphingolipids.

    “Pathways upregulated by this subcluster were involved in synthesis of several lipid subtypes, including glycosphingolipids and sphingolipids37 (Fig. 3F).”

    From The R136S mutation in the APOE3 gene confers resilience against tau pathology via inhibition of the cGAS-STING-IFN pathway (2025) · cites this paper as [37]

    Section 4, “The Role of Sphingolipids in Myelination” (page unavailable), says the sphingolipid metabolic pathway produces sphingolipids, including glycosphingolipids. The cited review does not report an OL2 subcluster or show that its lipid-synthesis pathways were upregulated; that finding appears to come from the citing paper’s Fig. 3F. (LLM, conf=0.85)

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Cited by

Sorted by citations

Authors
Giussani, P., Prinetti, A., & Tringali, C.
Affiliations
University of Milan (IT)
Corresponding author
  • Giussani, Paola
  • Tringali, Cristina
ORCID
Venue
Journal of Neurochemistry
Publisher
Wiley
Published
2020-08-25
Volume
156
Issue
4
Pages
403-414
ISSN
  • 0022-3042 (ISSN-L)
  • 1471-4159
DOI
10.1111/jnc.15133
OpenAlex
W3046064102
Cited by
91
References
3
Citations by year
2026: 18 · 2025: 23 · 2024: 12 · 2023: 14 · 2022: 17 · 2021: 6 · 2020: 1
FWCI
0.91
Type
journal article
Language
en
Open access
Licence
onlinelibrary.wiley.com/termsAndConditions#vor (vor)
Locations
  • Journal of Neurochemistry (publishedVersion)
  • PubMed (publishedVersion)
  • Archivio Istituzionale della Ricerca (Universita Degli Studi Di Milano) (submittedVersion, other-oa)
Topics
  • Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Sphingolipid Metabolism and Signaling
  • Health Sciences › Medicine › Physiology › Lysosomal Storage Disorders Research
  • Health Sciences › Medicine › Pathology and Forensic Medicine › Multiple Sclerosis Research Studies
Keywords
sphingolipids, myelination, myelin stability, demyelinating disease, multiple sclerosis, sphingolipidosis, sphingolipid metabolism, ceramide, dihydroceramide, sphingosine, dihydrosphingosine, glucosylceramide, galactosylceramide, brain development, plasma membrane organization
MeSH terms
Adult, Animals, Child, Demyelinating Diseases, Humans, Myelin Sheath, Nerve Fibers, Myelinated, Sphingolipids

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