The Role and Metabolism of Sulfatide in the Nervous System
Abstract
3-O-sulfogalactosylceramide or sulfatide is a major component of the myelin sheath in the central and peripheral nervous system. The examination of mice deficient in the sulfatide-synthesizing enzyme, cerebroside sulfotransferase, provided new insight into the role of sulfatide in the differentiation of myelinating cells, formation of the paranodal junction, and myelin maintenance. Although in general regarded as a marker for oligodendrocytes and Schwann cells, sulfatide is also present in astrocytes and neurons. The relatively low amount of sulfatide in neurons can dramatically increase in the absence of the sulfatide-degrading enzyme, arylsulfatase A, as in metachromatic leukodystrophy. Recent advance in the understanding of this disease comes from studies on new transgenic mouse models. Significant changes in sulfatide levels have also been observed more recently in Alzheimer's disease and other diseases, suggesting that sulfatide might be involved in the pathogenesis of these diseases as well. This review summarizes recent studies on the physiological and pathophysiological role of sulfatide using transgenic mice deficient in its synthesis or degradation.
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Lipid classes PS, PE, sulfatide, cerebroside and PA are important to cognitive functions.
“Brain lipidomics analysis indicates that apoE3-Jac expression increases several classes of lipids, such as PS, PE, sulfatide, cerebroside and PA, which are important to cognitive functions (38–40).”
From <i>APOE3</i> -Jacksonville (V236E) variant reduces self-aggregation and risk of dementia (2021) · cites this paper as [39]
Cites
Astrocytes synthesize apolipoprotein E and metabolize apolipoprotein E-containing lipoproteins
Pitas, R. E., Boyles, J. K., Lee, S. H., Foss, D., & Mahley, R. W. (1987) · Biochimica et Biophysica Acta (BBA) - Lipids and Lipid Metabolism · doi:10.1016/0005-2760(87)90295-5 · Cited by 693 · see citing works
Han, X., M. Holtzman, D., W. McKeel, D., Kelley, J., & Morris, J. C. (2002) · Journal of Neurochemistry · doi:10.1046/j.1471-4159.2002.00997.x · Cited by 626 · see citing works
Simons, M., Krämer, E.-M., Thiele, C., Stoffel, W., & Trotter, J. (2000) · The Journal of Cell Biology · doi:10.1083/jcb.151.1.143 · Cited by 293 · see citing works
TRAM, LAG1 and CLN8: members of a novel family of lipid-sensing domains?
Winter, E., & Ponting, C. P. (2002) · Trends in Biochemical Sciences · doi:10.1016/s0968-0004(02)02154-0 · Cited by 181 · see citing works
Enzyme, cell and gene‐based therapies for metachromatic leukodystrophy
Sevin, C., Aubourg, P., & Cartier, N. (2007) · Journal of Inherited Metabolic Disease · doi:10.1007/s10545-007-0540-z · Cited by 88 · see citing works
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van der Knaap, M. S., & Bugiani, M. (2017) · Acta Neuropathologica · doi:10.1007/s00401-017-1739-1 · Cited by 403 · see citing works
More than cholesterol transporters: lipoprotein receptors in CNS function and neurodegeneration
Lane-Donovan, C., Philips, G. T., & Herz, J. (2014) · Neuron · doi:10.1016/j.neuron.2014.08.005 · Cited by 158 · see citing works
Cholesterol: A Novel Regulatory Role in Myelin Formation
Saher, G., Quintes, S., & Nave, K.-A. (2011) · The Neuroscientist · doi:10.1177/1073858410373835 · Cited by 144 · see citing works
<i>APOE3</i> -Jacksonville (V236E) variant reduces self-aggregation and risk of dementia
Liu, C.-C., Murray, M. E., Li, X., Zhao, N., Wang, N., Heckman, M. G., Shue, F., Martens, Y., Li, Y., Raulin, A.-C., et al. (2021) · Science Translational Medicine · doi:10.1126/scitranslmed.abc9375 · Cited by 108 · see citing works
Bergner, C. G., van der Meer, F., Winkler, A., Wrzos, C., Türkmen, M., Valizada, E., Fitzner, D., Hametner, S., Hartmann, C., Pfeifenbring, S., et al. (2019) · Glia · doi:10.1002/glia.23598 · Cited by 94 · see citing works
“In addition, most sulfatides in the CNS are located in the myelin sheaths and oligodendrocytes (Eckhardt, 2008).”
BackgroundMembrane raft domains and remodeling in aging brain
Colin, J., Gregory-Pauron, L., Lanhers, M.-C., Claudepierre, T., Corbier, C., Yen, F. T., Malaplate-Armand, C., & Oster, T. (2016) · Biochimie · doi:10.1016/j.biochi.2016.08.014 · Cited by 55 · see citing works
Allende, M. L., Zhu, H., Kono, M., Hoachlander-Hobby, L. E., Huso, V. L., & Proia, R. L. (2021) · Cellular Signalling · doi:10.1016/j.cellsig.2020.109879 · Cited by 37 · see citing works
- Authors
- Eckhardt, M.
- Affiliations
- University of Bonn (DE)
- Corresponding author
- Eckhardt, Matthias
- ORCID
- Eckhardt, Matthias
- Venue
- Molecular Neurobiology
- Published
- 2008-05-09
- Volume
- 37
- Issue
- 2-3
- Pages
- 93-103
- ISSN
- 0893-7648 (ISSN-L)
- 1559-1182
- OpenAlex
- W2068475929
- Cited by
- 193
- References
- 5
- Citations by year
- 2026: 8 · 2025: 11 · 2024: 8 · 2023: 14 · 2022: 12 · 2021: 11 · 2020: 20 · 2019: 13 · 2018: 11 · 2017: 8 · 2016: 9 · 2015: 10 · 2014: 9 · 2013: 6 · 2012: 9
- FWCI
- 0.87
- Type
- journal article
- Language
- en
- Open access
- closed
- Licence
- www.springer.com/tdm (tdm)
- Locations
- Molecular Neurobiology (publishedVersion)
- PubMed (publishedVersion)
- 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
- sulfatide, myelin sheath, cerebroside sulfotransferase, arylsulfatase A, metachromatic leukodystrophy, paranodal junctions, myelin maintenance, oligodendrocytes, Schwann cells, astrocytes, neurons, Alzheimer's disease, transgenic mouse model
- MeSH terms
- Animals, Apolipoproteins E, Cell Differentiation, Galactosylceramides, Humans, Leukodystrophy, Metachromatic, Myelin Sheath, Nervous System, Neurons, Sulfoglycosphingolipids, Molecular Structure
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