Sulfatide
Sulfatides are a class of sulfated galactosylceramides synthesized primarily in the oligodendrocytes in the central nervous system. Sulfatides are a type of sulfolipid.[1][2] Sulfatides are thought to play a major role in myelin function and stability, as evidenced by the fact that mice lacking the enzyme necessary to produce sulfatides during myelin sheath formation have thinner myelin sheaths, and experience progressive hindlimb paralysis as a result of demyelination of their lower spinal cords.[3]
Clinical significance
Sulfatidosis, or metachromatic leukodystrophy, is a lysosomal storage disease characterized by accumulation of sulfatides due to an inability to degrade them in lysosomes.[4]
Role in Alzheimer's disease
Alterations in sulfatide metabolism, trafficking, and homeostasis are present in the earliest clinically recognizable stages of Alzheimer's disease.
Relationship to vitamin K
Several studies have demonstrated a relationship between vitamin K and sufatides. After mice were administered warfarin to decreased their brain sulfatide levels, vitamin K administration was able to restore these levels to normal.[5][6]
References
- ↑ Michael A Lieberman, Rick Ricer (2009). Lippincott's Illustrated Q&A Review of Biochemistry (1 Pap/Psc ed ed.). Lippincott Williams & Wilkins. ISBN 1-60547-302-2.
- ↑ Huang JQ, Trasler JM, Igdoura S, Michaud J, Hanal N, Gravel RA (October 1997). "Apoptotic cell death in mouse models of GM2 gangliosidosis and observations on human Tay-Sachs and Sandhoff diseases". Human Molecular Genetics 6 (11): 1879–85. doi:10.1093/hmg/6.11.1879. PMID 9302266.
- ↑ Coetzee, T.; Fujita, N.; Dupree, J.; Shi, R.; Blight, A.; Suzuki, K.; Suzuki, K.; Popko, B. (1996). "Myelination in the Absence of Galactocerebroside and Sulfatide: Normal Structure with Abnormal Function and Regional Instability". Cell 86 (2): 209–219. doi:10.1016/S0092-8674(00)80093-8. PMID 8706126.
- ↑ Whitfield, P. D.; Sharp, P. C.; Johnson, D. W.; Nelson, P.; Meikle, P. J. (2001). "Characterization of Urinary Sulfatides in Metachromatic Leukodystrophy Using Electrospray Ionization-Tandem Mass Spectrometry". Molecular Genetics and Metabolism 73 (1): 30–37. doi:10.1006/mgme.2001.3165. PMID 11350180.
- ↑ Sundaram, K. S.; Lev, M. (1990). "Regulation of sulfotransferase activity by vitamin K in mouse brain". Archives of Biochemistry and Biophysics 277 (1): 109–113. doi:10.1016/0003-9861(90)90557-F. PMID 1968327.
- ↑ Shearer, M. J.; Newman, P. (2008). "Metabolism and cell biology of vitamin K". Thrombosis and Haemostasis. doi:10.1160/TH08-03-0147.
External links
- Sulfatides at the US National Library of Medicine Medical Subject Headings (MeSH)
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