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The Role Of Sphingolipids In Myelination And Myelin Stability And Their Involvement In Childhood And Adult Demyelinating Disorders

P. Giussani, A. Prinetti, C. Tringali
Published 2020 · Biology, Medicine

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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.
This paper references
10.1055/s-2006-956758
Multiple sclerosis.
W. L. Benedict (1954)
[Multiple sclerosis; review].
T. Fog (1954)
10.1016/s0021-9258(18)69582-1
The enzymatic synthesis of psychosine.
W. Cleland (1960)
The enzymatic synthesis of psy - chosine
R. P. Coelho (1960)
10.1016/S0140-6736(68)92510-5
Sphingolopids and phospholipids of myelin in multiple sclerosis.
J. Cumings (1968)
10.1111/j.1365-2990.1979.tb00639.x
THE FRAGILITY OF CEREBRAL LYSOSOMES IN MULTIPLE SCLEROSIS
S. McKeown (1979)
10.1016/0022-510X(80)90076-3
Anti-ganglioside antibodies in multiple sclerosis
R. Arnon (1980)
10.1016/0022-510X(82)90191-5
A change in the cerebrosides and sulfatides in a demyelinating nervous system Development of the methodology and study of multiple sclerosis and Wallerian degeneration
S. Yahara (1982)
10.1126/SCIENCE.6719111
Progressive accumulation of toxic metabolite in a genetic leukodystrophy.
H. Igisu (1984)
10.1016/0022-510X(86)90048-1
Anti-glycolipid antibodies and their immune complexes in multiple sclerosis
N. Kasai (1986)
10.1016/0009-8981(89)90224-6
Regulation of protein kinase C by sphingosine and lysosphingolipids.
Y. Hannun (1989)
10.1159/000112165
Phenotypic consequences of low arylsulfatase A genotypes (ASAp/ASAp and ASA-/ASAp): does there exist an association with multiple sclerosis?
J. Kappler (1991)
10.1016/S0092-8674(00)80093-8
Myelination in the Absence of Galactocerebroside and Sulfatide: Normal Structure with Abnormal Function and Regional Instability
T. Coetzee (1996)
10.1093/HMG/6.10.1693
Genetics of Multiple Sclerosis
D. Dyment (1997)
10.1002/(SICI)1097-0029(19980601)41:5<431::AID-JEMT9>3.0.CO;2-S
Galactolipids in the formation and function of the myelin sheath
J. Dupree (1998)
10.1074/jbc.273.32.20354
Cytokine-mediated Induction of Ceramide Production Is Redox-sensitive
I. Singh (1998)
10.1177/135245859900500i603
Multiple sclerosis is associated with enhanced B cell responses to the ganglioside GD I a
S. Matà (1999)
10.1016/S1388-1981(99)00201-2
Analysis of sulfatide from rat cerebellum and multiple sclerosis white matter by negative ion electrospray mass spectrometry.
B. Marbois (2000)
10.1016/S0300-9084(01)01308-6
Brain gangliosides: functional ligands for myelin stability and the control of nerve regeneration.
A. Vyas (2001)
10.1523/JNEUROSCI.22-15-06507.2002
A Myelin Galactolipid, Sulfatide, Is Essential for Maintenance of Ion Channels on Myelinated Axon But Not Essential for Initial Cluster Formation
T. Ishibashi (2002)
10.1073/pnas.072211699
Gangliosides are functional nerve cell ligands for myelin-associated glycoprotein (MAG), an inhibitor of nerve regeneration
A. Vyas (2002)
10.1016/S0165-5728(03)00131-0
Antibodies to sulfatide in cerebrospinal fluid of patients with multiple sclerosis
A. A. Ilyas (2003)
10.1016/S0967-5868(02)00270-9
Increased circulating T cell reactivity to GM3 and GQ1b gangliosides in primary progressive multiple sclerosis
M. Pender (2003)
10.1007/BF00286589
Arylsulfatase A in pseudodeficiency
B. Herz (2004)
10.1023/A:1011027125744
Serum Ganglioside Patterns in Multiple Sclerosis
E. Zaprianova (2004)
10.1016/J.SEMCDB.2004.03.002
Secondary accumulation of gangliosides in lysosomal storage disorders.
S. Walkley (2004)
10.1002/glia.10327
Sulfatide is a negative regulator of oligodendrocyte differentiation: Development in sulfatide‐null mice
Y. Hirahara (2004)
10.1111/j.1471-4159.2005.03451.x
Novel role of sphingosine kinase 1 as a mediator of neurotrophin‐3 action in oligodendrocyte progenitors
H. Saini (2005)
10.1191/135248506ms1279oa
Anti-GD2-like IgM autoreactivity in multiple sclerosis patients
S. Marconi (2006)
10.1038/nm1344
Lipid microarrays identify key mediators of autoimmune brain inflammation
J. L. Kanter (2006)
10.1007/BF02531040
Gas liquid chromatographic analysis of sphingosine bases in sphingolipids of human normal and multiple sclerosis cerebral white matter
E. A. Moscatelli (2006)
10.1194/jlr.M600084-JLR200
Krabbe disease: psychosine-mediated activation of phospholipase A2 in oligodendrocyte cell death Published, JLR Papers in Press, April 27, 2006.
S. Giri (2006)
10.1007/s11064-006-9145-4
Mini Review: Immune Response to Myelin-Derived Sulfatide and CNS-Demyelination
R. Halder (2006)
10.1124/jpet.107.123927
The Immunomodulator FTY720 Has a Direct Cytoprotective Effect in Oligodendrocyte Progenitors
Rochelle P Coelho (2007)
10.1007/s10545-007-0632-9
The pathogenesis and treatment of acid sphingomyelinase-deficient Niemann–Pick disease
E. Schuchman (2007)
10.1002/glia.20576
Functional consequences of S1P receptor modulation in rat oligodendroglial lineage cells
C. Jung (2007)
10.1002/ana.21227
FTY720 modulates human oligodendrocyte progenitor process extension and survival
V. Miron (2008)
10.1074/jbc.M803301200
Integrin-associated Lyn Kinase Promotes Cell Survival by Suppressing Acid Sphingomyelinase Activity*
D. Chudakova (2008)
10.4049/jimmunol.181.11.8077
Sulfatide, A Major Lipid Component of Myelin Sheath, Activates Inflammatory Responses As an Endogenous Stimulator in Brain-Resident Immune Cells1
Sae-Bom Jeon (2008)
10.2353/ajpath.2008.080478
Cyclical and dose-dependent responses of adult human mature oligodendrocytes to fingolimod.
V. Miron (2008)
10.1093/brain/awn190
A defect of sphingolipid metabolism modifies the properties of normal appearing white matter in multiple sclerosis
David Wheeler (2008)
10.1038/ng.396
Genome-wide association study identifies new multiple sclerosis susceptibility loci on chromosomes 12 and 20
M. Bahlo (2009)
10.1017/S0317167100007940
Adult-Type Metachromatic Leukodystrophy Mimicking Multiple Sclerosis
S. Chebel (2009)
10.1177/1352458509106708
Molecular and immunogenic features of myelin lipids: incitants or modulators of multiple sclerosis?
M. Podbielska (2009)
10.1038/ng.401
Meta-analysis of genome scans and replication identify CD6, IRF8 and TNFRSF1A as new multiple sclerosis susceptibility loci
P. D. Jager (2009)
10.1093/hmg/ddp120
Pathway and network-based analysis of genome-wide association studies in multiple sclerosis
S. Baranzini (2009)
10.1523/JNEUROSCI.5597-08.2009
Psychosine Accumulates in Membrane Microdomains in the Brain of Krabbe Patients, Disrupting the Raft Architecture
A. White (2009)
10.1016/j.febslet.2009.10.011
Brain gangliosides in axon–myelin stability and axon regeneration
R. Schnaar (2010)
10.1093/brain/awq192
Genetic variation influences glutamate concentrations in brains of patients with multiple sclerosis.
S. Baranzini (2010)
10.1007/s12017-010-8128-4
Sphingolipids in Multiple Sclerosis
Arundhati Jana (2010)
10.1097/WNF.0b013e3181cbf825
Mechanism of Action of Oral Fingolimod (FTY720) in Multiple Sclerosis
J. Chun (2010)
10.1007/s11064-010-0380-3
Secondary Alterations of Sphingolipid Metabolism in Lysosomal Storage Diseases
A. Prinetti (2010)
10.1016/j.ajhg.2010.01.017
Genome-wide association study in a high-risk isolate for multiple sclerosis reveals associated variants in STAT3 gene.
E. Jakkula (2010)
10.1016/j.prostaglandins.2009.04.002
Sphingosine-1-phosphate and oligodendrocytes: from cell development to the treatment of multiple sclerosis.
Rochelle P Coelho (2010)
Genome - wide association study in a high - risk isolate for multiple sclerosis reveals associated variants in STAT 3 gene
A. Jana (2010)
10.1016/J.YNEU.2011.03.010
Genetic variation influences glutamate concentrations in brains of patients with multiple sclerosis
A. Minagar (2011)
10.1038/nature10251
Genetic risk and a primary role for cell-mediated immune mechanisms in multiple sclerosis
S. Sawcer (2011)
10.1021/cr2002917
Sphingolipid and Glycosphingolipid Metabolic Pathways in the Era of Sphingolipidomics
A. Merrill (2011)
10.1096/fj.10-173203
Neurobiological effects of sphingosine 1‐phosphate receptor modulation in the cuprizone model
H. Kim (2011)
10.1016/j.bbadis.2010.09.017
Genetics of multiple sclerosis.
I. Hoppenbrouwers (2011)
Arylsulfatase a gene polymorphisms in relapsing remitting multiple sclerosis: genotype-phenotype correlation and estimation of disease progression.
K. B. Baronica (2011)
10.1002/ana.22609
Genome‐wide meta‐analysis identifies novel multiple sclerosis susceptibility loci
N. Patsopoulos (2011)
10.1177/1352458512439118
A genome-wide association study in progressive multiple sclerosis
F. Martinelli-Boneschi (2012)
10.1016/j.spen.2012.10.001
Relative incidence of inherited white matter disorders in childhood to acquired pediatric demyelinating disorders.
A. Vanderver (2012)
10.1111/j.1600-0404.2011.01554.x
Myelin glycosphingolipid immunoreactivity and CSF levels in multiple sclerosis
S. Haghighi (2012)
10.1007/s10545-012-9509-7
Disorders of phospholipids, sphingolipids and fatty acids biosynthesis: toward a new category of inherited metabolic diseases
F. Lamari (2012)
10.1194/jlr.R026682
Role of sulfatide in normal and pathological cells and tissues
Tadanobu Takahashi (2012)
10.1093/hmg/ddt190
Missense mutation in mouse GALC mimics human gene defect and offers new insights into Krabbe disease.
G. Potter (2013)
10.1371/journal.pone.0055149
PDGF is Required for Remyelination-Promoting IgM Stimulation of Oligodendrocyte Progenitor Cell Proliferation
J. Watzlawik (2013)
10.1111/jnc.12341
Levels of plasma sulfatides C18 : 0 and C24 : 1 correlate with disease status in relapsing–remitting multiple sclerosis
A. L. Moyano (2013)
10.1038/ng.2770
Analysis of immune-related loci identifies 48 new susceptibility variants for multiple sclerosis
A. Beecham (2013)
10.1016/j.jns.2013.01.007
Increased CSF sulfatide levels and serum glycosphingolipid antibody levels in healthy siblings of multiple sclerosis patients
S. Haghighi (2013)
10.1016/j.bbalip.2013.11.001
Second generation S1P pathway modulators: research strategies and clinical developments.
M. Bigaud (2014)
10.1016/S1474-4422(14)70041-9
Multiple sclerosis genetics
S. Sawcer (2014)
10.1093/brain/awu139
Cerebrospinal fluid ceramides from patients with multiple sclerosis impair neuronal bioenergetics.
Ó. G. Vidaurre (2014)
10.1523/JNEUROSCI.0885-14.2014
Brain Glycolipids Suppress T Helper Cells and Inhibit Autoimmune Demyelination
M. Mycko (2014)
10.1159/000362988
Sphingolipids: Important Players in Multiple Sclerosis
R. Halmer (2014)
10.1016/j.bbadis.2014.01.008
Molecular cloning and knockdown of galactocerebrosidase in zebrafish: new insights into the pathogenesis of Krabbe's disease.
D. Zizioli (2014)
10.1038/nm.3681
Regulation of astrocyte activation by glycolipids drives chronic CNS inflammation
Lior Mayo (2014)
10.3390/ijms15034356
Sphingolipids: Key Regulators of Apoptosis and Pivotal Players in Cancer Drug Resistance
P. Giussani (2014)
10.4049/jimmunol.1302898
Dendritic Cells and Anergic Type I NKT Cells Play a Crucial Role in Sulfatide-Mediated Immune Regulation in Experimental Autoimmune Encephalomyelitis
I. Maričić (2014)
Molecular cloning and knockdown of ga - lactocerebrosidase in zebrafish : New insights into the pathogenesis of Krabbe ' s disease
D. Zizioli (2014)
Krabbe disease : Psychosine - mediated activation of phospholipase A 2 in oligodendrocyte cell death
P. Giussani (2014)
10.1016/j.bbalip.2014.12.016
Metabolism and functions of lipids in myelin.
S. Schmitt (2015)
10.1093/brain/awu397
Differential diagnosis of Mendelian and mitochondrial disorders in patients with suspected multiple sclerosis.
J. Weisfeld-Adams (2015)
10.1111/tra.12404
Molecular Mechanisms of Disease Pathogenesis Differ in Krabbe Disease Variants
S. J. Spratley (2016)
10.1002/jnr.23751
Beyond Krabbe's disease: The potential contribution of galactosylceramidase deficiency to neuronal vulnerability in late‐onset synucleinopathies
Michael S. Marshall (2016)
10.1002/glia.22949
S1P1 deletion in oligodendroglial lineage cells: Effect on differentiation and myelination
D. Dukala (2016)
10.1111/jnc.13385
The relationship between glucocerebrosidase mutations and Parkinson disease
A. Migdalska‐Richards (2016)
10.1007/s00439-017-1784-9
Common genetic etiology between “multiple sclerosis-like” single-gene disorders and familial multiple sclerosis
A. Traboulsee (2017)
10.1371/journal.pone.0178103
Psychosine enhances the shedding of membrane microvesicles: Implications in demyelination in Krabbe’s disease
L. D'auria (2017)
10.1136/bcr-2017-220274
Fabry heterozygote mimicking multiple sclerosis
W. Y. Yau (2017)
10.1371/journal.pone.0178622
Acid sphingomyelinase deficiency enhances myelin repair after acute and chronic demyelination
Marwan Chami (2017)
10.1093/hmg/ddx153
Heterozygote galactocerebrosidase (GALC) mutants have reduced remyelination and impaired myelin debris clearance following demyelinating injury
Nicole Scott-Hewitt (2017)
10.1016/j.tig.2017.09.004
The Genetics of Multiple Sclerosis: From 0 to 200 in 50 Years.
S. Baranzini (2017)
10.3390/molecules22030344
Sphingosine 1-Phosphate Receptor 1 Signaling in Mammalian Cells
N. Pyne (2017)
10.1159/000484621
Blockade of Experimental Multiple Sclerosis by Inhibition of the Acid Sphingomyelinase/Ceramide System
K. A. Becker (2017)
10.1038/s41467-017-02361-y
The mechanism of glycosphingolipid degradation revealed by a GALC-SapA complex structure
C. Hill (2017)
10.13188/2332-3469.1000035
Diverse Biological Functions of Sphingolipids in the CNS: Ceramide and Sphingosine Regulate Myelination in Developing Brain but Stimulate Demyelination during Pathogenesis of Multiple Sclerosis
S. Dasgupta (2017)
10.1098/rsob.170069
Sphingolipids: membrane microdomains in brain development, function and neurological diseases
Anne S B Olsen (2017)
10.4103/1673-5374.228712
Heterozygous carriers of galactocerebrosidase mutations that cause Krabbe disease have impaired microglial function and defective repair of myelin damage
Nicole Scott-Hewitt (2018)
10.1101/cshperspect.a028936
Multiple Sclerosis Pathology.
H. Lassmann (2018)
10.1002/cti2.1018
Genome‐wide association studies of multiple sclerosis
C. Cotsapas (2018)
10.1002/9781119013112.CH29
Sphingolipidoses and Related Disorders
A. Laquérriere (2018)
10.18632/oncotarget.23970
Fabry disease and multiple sclerosis misdiagnosis: the role of family history and neurological signs
P. Colomba (2018)
10.1038/s41598-018-21497-5
Enhanced release of acid sphingomyelinase-enriched exosomes generates a lipidomics signature in CSF of Multiple Sclerosis patients
D. Pieragostino (2018)
10.1016/j.cell.2018.09.049
Low-Frequency and Rare-Coding Variation Contributes to Multiple Sclerosis Risk
Mitja Nikolaos A. Ashley H. Theresa An Bénédicte Marie B Mitrovič Patsopoulos Beecham Dankowski Goris Duboi (2018)
Sphingolipidoses and Related Disorders. Developmental Neuropathology, Second Edition
A Laquerriere (2018)
Multiple sclerosis pathology. Cold Spring Harbor Perspectives in Medicine
H Lassmann (2018)
10.1111/jnc.14540
Sphingolipids and neuronal degeneration in lysosomal storage disorders
Sara Grassi (2019)
10.1016/j.jbior.2018.11.002
Intra- and intercellular trafficking in sphingolipid metabolism in myelination.
B. Wattenberg (2019)
10.3389/fphar.2019.00807
Sphingosine 1-Phosphate Receptors and Metabolic Enzymes as Druggable Targets for Brain Diseases
Sara Grassi (2019)
10.33594/000000183
Pharmacological Inhibition of Acid Sphingomyelinase Ameliorates Experimental Autoimmune Encephalomyelitis.
S. Walter (2019)
10.3390/ijms20133304
The Link between Gaucher Disease and Parkinson’s Disease Sheds Light on Old and Novel Disorders of Sphingolipid Metabolism
Rossella Indellicato (2019)
10.1111/ene.13819
Multiple sclerosis – a review
R. Dobson (2019)
10.1186/s12944-019-0965-z
Role of cholesterol and sphingolipids in brain development and neurological diseases
Ghulam Hussain (2019)
10.1016/j.intimp.2019.105923
Combination of Imipramine, a sphingomyelinase inhibitor, and β-caryophyllene improve their therapeutic effects on experimental autoimmune encephalomyelitis (EAE).
V. Askari (2019)
10.7554/eLife.51067
The Ormdl genes regulate the sphingolipid synthesis pathway to ensure proper myelination and neurologic function in mice
Benjamin A. Clarke (2019)
10.1016/j.nbd.2018.11.018
Inactivation of sphingosine-1-phosphate receptor 2 (S1PR2) decreases demyelination and enhances remyelination in animal models of multiple sclerosis
M. Seyedsadr (2019)
10.1194/jlr.TR119000427
Lipid rafts and neurodegeneration: structural and functional roles in physiologic aging and neurodegenerative diseases
Sara Grassi (2019)
10.1073/pnas.1912108116
Genetic ablation of acid ceramidase in Krabbe disease confirms the psychosine hypothesis and identifies a new therapeutic target
Yedda Li (2019)
10.1007/s00401-019-02049-1
Detrimental and protective action of microglial extracellular vesicles on myelin lesions: astrocyte involvement in remyelination failure
Marta Lombardi (2019)
10.1523/JNEUROSCI.2095-18.2018
Glial Sulfatides and Neuronal Complex Gangliosides Are Functionally Interdependent in Maintaining Myelinating Axon Integrity
R. McGonigal (2019)
10.1194/jlr.S091827
A perilous path: the inborn errors of sphingolipid metabolism
T. Dunn (2019)
10.1016/j.msard.2018.11.024
Acid sphingomyelinase: No potential as a biomarker for multiple sclerosis.
C.E. Leurs (2019)
The role of Sphingolipids in myelination and myelin stability and their involvement in childhood and adult demyelinating disorders
P Giussani (2021)
Anti - GD 2 - like IgM autoreactivity in multiple sclerosis patients
S. Marconi



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