Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress
Clinical Collections
Advances in Alzheimer’s

Keynote webinar | Spotlight on medication adherence

LIVE: Thursday 27th June 2024, 18:00-19:30 (CEST)
Join our expert panel to discover why you need to understand the drivers of non-adherence in your patients, and how you can optimize medication adherence in your clinics to drastically improve patient outcomes.
ADVANCED SEARCH
Log in
Top
NeuroMolecular Medicine
Published in: NeuroMolecular Medicine 1/2021

01-03-2021 | Gaucher Disease | Review Paper

Sphingolipids as Regulators of Neuro-Inflammation and NADPH Oxidase 2

Authors: Emma J. Arsenault, Colin M. McGill, Brian M. Barth

Published in: NeuroMolecular Medicine | Issue 1/2021

Login to get access

Abstract

Neuro-inflammation accompanies numerous neurological disorders and conditions where it can be associated with a progressive neurodegenerative pathology. In a similar manner, alterations in sphingolipid metabolism often accompany or are causative features in degenerative neurological conditions. These include dementias, motor disorders, autoimmune conditions, inherited metabolic disorders, viral infection, traumatic brain and spinal cord injury, psychiatric conditions, and more. Sphingolipids are major regulators of cellular fate and function in addition to being important structural components of membranes. Their metabolism and signaling pathways can also be regulated by inflammatory mediators. Therefore, as certain sphingolipids exert distinct and opposing cellular roles, alterations in their metabolism can have major consequences. Recently, regulation of bioactive sphingolipids by neuro-inflammatory mediators has been shown to activate a neuronal NADPH oxidase 2 (NOX2) that can provoke damaging oxidation. Therefore, the sphingolipid-regulated neuronal NOX2 serves as a mechanistic link between neuro-inflammation and neurodegeneration. Moreover, therapeutics directed at sphingolipid metabolism or the sphingolipid-regulated NOX2 have the potential to alleviate neurodegeneration arising out of neuro-inflammation.
Literature
Abou Daher, A., Francis, M., Azzam, P., Ahmad, A., Eid, A. A., Fornoni, A., et al. (2020). Modulation of radiation-induced damage of human glomerular endothelial cells by SMPDL3B. FASEB Journal, 34(6), 7915–7926.PubMedCrossRef
Alayoubi, A. M., Wang, J. C., Au, B. C., Carpentier, S., Garcia, V., Dworski, S., et al. (2013). Systemic ceramide accumulation leads to severe and varied pathological consequences. EMBO Molecular Medicine, 5(6), 827–842.PubMedPubMedCentralCrossRef
Alpaugh, M., Galleguillos, D., Forero, J., Morales, L. C., Lackey, S. W., Kar, P., et al. (2017). Disease-modifying effects of ganglioside GM1 in Huntington’s disease models. EMBO Molecular Medicine, 9(11), 1537–1557.PubMedPubMedCentralCrossRef
Annunziata, I., Bouchè, V., Lombardi, A., Settembre, C., & Ballabio, A. (2007). Multiple sulfatase deficiency is due to hypomorphic mutations of the SUMF1 gene. Human Mutation, 28(9), 928–928.PubMedCrossRef
Ashe, K. M., Budman, E., Bangari, D. S., Siegel, C. S., Nietupski, J. B., Wang, B., et al. (2015). Efficacy of enzyme and substrate reduction therapy with a novel antagonist of glucosylceramide synthase for Fabry disease. Molecular Medicine, 21(1), 389–399.PubMedPubMedCentralCrossRef
Bandaru, V. V., McArthur, J. C., Sacktor, N., Cutler, R. G., Knapp, E. L., Mattson, M. P., & Haughey, N. J. (2007). Associative and predictive biomarkers of dementia in HIV-1–infected patients. Neurology, 68(18), 1481–1487.PubMedCrossRef
Bandaru, V. V. R., Mielke, M. M., Sacktor, N., McArthur, J. C., Grant, I., Letendre, S., et al. (2013). A lipid storage–like disorder contributes to cognitive decline in HIV-infected subjects. Neurology, 81(17), 1492–1499.PubMedPubMedCentralCrossRef
Bang, J., Spina, S., & Miller, B. L. (2015). Frontotemporal dementia. The Lancet, 386(10004), 1672–1682.CrossRef
Bansal, R., Winkler, S., & Bheddah, S. (1999). Negative regulation of oligodendrocyte differentiation by galactosphingolipids. Journal of Neuroscience, 19(18), 7913–7924.PubMedCrossRef
Bär, J., Linke, T., Ferlinz, K., Neumann, U., Schuchman, E. H., & Sandhoff, K. (2001). Molecular analysis of acid ceramidase deficiency in patients with Farber disease. Human Mutation, 17(3), 199–209.PubMedCrossRef
Barth, B. M., Cabot, M. C., & Kester, M. (2011). Ceramide-based therapeutics for the treatment of cancer. Anti-Cancer Agents in Medicinal Chemistry, 11(9), 911–919.PubMedCrossRef
Barth, B. M., Gustafson, S. J., Hankins, J. L., Kaiser, J. M., Haakenson, J. K., Kester, M., et al. (2012b). Ceramide kinase regulates TNFα-stimulated NADPH oxidase activity and eicosanoid biosynthesis in neuroblastoma cells. Cellular Signaling, 24(6), 1126–1133.CrossRef
Barth, B. M., Gustafson, S. J., & Kuhn, T. B. (2012a). Neutral sphingomyelinase activation precedes NADPH oxidase-dependent damage in neurons exposed to the proinflammatory cytokine tumor necrosis factor-α. Journal of Neuroscience Research, 90(1), 229–242.PubMedCrossRef
Barth, B. M., Gustafson, S. J., Young, M. M., Fox, T. E., Shanmugavelandy, S. S., Kaiser, J. M., et al. (2010). Inhibition of NADPH oxidase by glucosylceramide confers chemoresistance. Cancer Biology and Therapy, 10(11), 1126–1136.PubMedCrossRefPubMedCentral
Barth, B. M., Shanmugavelandy, S. S., Kaiser, J. M., McGovern, C., Altınoğlu, E. İ, Haakenson, J. K., et al. (2013). PhotoImmunoNanoTherapy reveals an anticancer role for sphingosine kinase 2 and dihydrosphingosine-1-phosphate. ACS Nano, 7(3), 2132–2144.PubMedPubMedCentralCrossRef
Barth, B. M., Stewart-Smeets, S., & Kuhn, T. B. (2009). Proinflammatory cytokines provoke oxidative damage to actin in neuronal cells mediated by Rac1 and NADPH oxidase. Molecular and Cellular Neuroscience, 41(2), 274–285.PubMedCrossRef
Becker, K. A., Uerschels, A. K., Goins, L., Doolen, S., McQuerry, K. J., Bielawski, J., et al. (2020). Role of 1-Deoxysphingolipids in docetaxel neurotoxicity. Journal of Neurochemistry, 154(6), 662–672.PubMedCrossRefPubMedCentral
Bedard, K., & Krause, K.-H. (2007). The NOX family of ROS-generating NADPH oxidases: Physiology and pathophysiology. Physiology Reviews, 87(1), 245–313.CrossRef
Bejaoui, K., Uchida, Y., Yasuda, S., Ho, M., Nishijima, M., Brown, R. H., et al. (2002). Hereditary sensory neuropathy type 1 mutations confer dominant negative effects on serine palmitoyltransferase, critical for sphingolipid synthesis. The Journal of Clinical Investigation, 110(9), 1301–1308.PubMedPubMedCentralCrossRef
Bembi, B., Marchetti, F., Guerci, V. I., Ciana, G., Addobbati, R., Grasso, D., et al. (2006). Substrate reduction therapy in the infantile form of Tay-Sachs disease. Neurology, 66(2), 278–280.PubMedCrossRef
Biancini, G. B., Vanzin, C. S., Rodrigues, D. B., Deon, M., Ribas, G. S., Barschak, A. G., et al. (2012). Globotriaosylceramide is correlated with oxidative stress and inflammation in Fabry patients treated with enzyme replacement therapy. Biochimica et Biophysica Acta (BBA)—Molecular Basis of Disease, 1822(2), 226–232.CrossRef
Bickert, A., Ginkel, C., Kol, M., Vom Dorp, K., Jastrow, H., Degen, J., et al. (2015). Functional characterization of enzymes catalyzing ceramide phosphoethanolamine biosynthesis in mice. Journal of Lipid Research, 56(4), 821–835.PubMedPubMedCentralCrossRef
Blaho, V. A., Galvani, S., Engelbrecht, E., Liu, C., Swendeman, S. L., Kono, M., et al. (2015). HDL-bound sphingosine-1-phosphate restrains lymphopoiesis and neuroinflammation. Nature, 523(7560), 342–346.PubMedPubMedCentralCrossRef
Blomqvist, M., Borén, J., Zetterberg, H., Blennow, K., Månsson, J. E., & Ståhlman, M. (2017). High-throughput analysis of sulfatides in cerebrospinal fluid using automated extraction and UPLC-MS/MS. Journal of Lipid Research, 58(7), 1482–1489.PubMedPubMedCentralCrossRef
Bode, H., Bourquin, F., Suriyanarayanan, S., Wei, Y., Alecu, I., Othman, A., et al. (2016). HSAN1 mutations in serine palmitoyltransferase reveal a close structure-function-phenotype relationship. Human Molecular Genetics, 25(5), 853–865.PubMedCrossRef
Boudker, O., & Futerman, A. H. (1993). Detection and characterization of ceramide-1-phosphate phosphatase activity in rat liver plasma membrane. Journal of Biological Chemistry, 268(29), 22150–22155.CrossRefPubMed
Breiden, B., & Sandhoff, K. (2020). Mechanism of secondary ganglioside and lipid accumulation in Lysosomal disease. International Journal of Molecular Sciences, 21(7), 2566.PubMedCentralCrossRef
Brekk, O. R., Korecka, J. A., Crapart, C. C., Huebecker, M., MacBain, Z. K., Rosenthal, S. A., et al. (2020). Upregulating β-hexosaminidase activity in rodents prevents α-synuclein lipid associations and protects dopaminergic neurons from α-synuclein-mediated neurotoxicity. Acta Neuropathologica Communications, 8(1), 127.PubMedPubMedCentralCrossRef
Bright, F., Werry, E. L., Dobson-Stone, C., Piguet, O., Ittner, L. M., Halliday, G. M., et al. (2019). Neuroinflammation in frontotemporal dementia. Nature Reviews Neurology, 15(9), 540–555.PubMedCrossRef
Brinkmann, V., Davis, M. D., Heise, C. E., Albert, R., Cottens, S., Hof, R., et al. (2002). The immune modulator FTY720 targets sphingosine 1-phosphate receptors. Journal of Biological Chemistry, 277(24), 21453–21457.CrossRefPubMed
Brück, W. (2005). The pathology of multiple sclerosis is the result of focal inflammatory demyelination with axonal damage. Journal of neurology, 252(5), v3–v9.PubMedCrossRef
Cabukusta, B., Nettebrock, N. T., Kol, M., Hilderink, A., Tafesse, F. G., & Holthuis, J. (2017). Ceramide phosphoethanolamine synthase SMSr is a target of caspase-6 during apoptotic cell death. Bioscience Reports, 37(4), 20170867.CrossRef
Chen, H., Chan, A. Y., Stone, D. U., & Mandal, N. A. (2014). Beyond the cherry-red spot: Ocular manifestations of sphingolipid-mediated neurodegenerative and inflammatory disorders. Survey of Ophthalmology, 59(1), 64–76.PubMedCrossRef
Cheng, H., Wang, M., Li, J. L., Cairns, N. J., & Han, X. (2013). Specific changes of sulfatide levels in individuals with pre-clinical Alzheimer’s disease: An early event in disease pathogenesis. Journal of Neurochemistry, 127(6), 733–738.PubMedCrossRef
Choi, S. H., Aid, S., Kim, H. W., Jackson, S. H., & Bosetti, F. (2012). Inhibition of NADPH oxidase promotes alternative and anti-inflammatory microglial activation during neuroinflammation. Journal of Neurochemistry, 120(2), 292–301.PubMedCrossRef
Cologna, S. M., Cluzeau, C. V., Yanjanin, N. M., Blank, P. S., Dail, M. K., Siebel, S., et al. (2014). Human and mouse neuroinflammation markers in Niemann-Pick disease, type C1. Journal of Inherited Metabolic Disease, 37(1), 83–92.PubMedCrossRef
Constantin, G., Laudanna, C., Baron, P., & Berton, G. (1994). Sulfatides trigger cytokine gene expression and secretion in human monocytes. FEBS Letters, 350(1), 66–70.PubMedCrossRef
Cristóvão, A. C., Guhathakurta, S., Bok, E., Je, G., Yoo, S. D., Choi, D. H., & Kim, Y. S. (2012). NADPH oxidase 1 mediates α-synucleinopathy in Parkinson’s disease. Journal of Neuroscience, 32(42), 14465–14477.PubMedCrossRef
Cutler, R. G., Pedersen, W. A., Camandola, S., Rothstein, J. D., & Mattson, M. P. (2002). Evidence that accumulation of ceramides and cholesterol esters mediates oxidative stress–induced death of motor neurons in amyotrophic lateral sclerosis. Annals of Neurology: Official Journal of the American Neurological Association and the Child Neurology Society, 52(4), 448–457.CrossRef
Czubowicz, K., Jęśko, H., Wencel, P., Lukiw, W. J., & Strosznajder, R. P. (2019). The role of ceramide and sphingosine-1-phosphate in Alzheimer’s disease and other neurodegenerative disorders. Molecular Neurobiology, 56(8), 5436–5455.PubMedPubMedCentralCrossRef
D’Angelo, G., Capasso, S., Sticco, L., & Russo, D. (2013). Glycosphingolipids: Synthesis and functions. The FEBS Journal, 280(24), 6338–6353.PubMedCrossRef
De Wit, N. M., den Hoedt, S., Martinez-Martinez, P., Rozemuller, A. J., Mulder, M. T., & de Vries, H. E. (2019). Astrocytic ceramide as possible indicator of neuroinflammation. Journal of Neuroinflammation, 16(48), 1–11.
Di Pardo, A., Amico, E., Basit, A., Armirotti, A., Joshi, P., Neely, M. D., & Pepe, G. (2017a). Defective sphingosine-1-phosphate metabolism is a druggable target in Huntington’s disease. Scientific Reports, 7(1), 1–14.CrossRef
Di Pardo, A., Basit, A., Armirotti, A., Amico, E., Castaldo, S., Pepe, G., et al. (2017b). De novo synthesis of sphingolipids is defective in experimental models of Huntington’s disease. Frontiers in Neuroscience, 11, 698.PubMedPubMedCentralCrossRef
Di Pardo, A., Castaldo, S., Amico, E., Pepe, G., Marracino, F., Capocci, L., et al. (2018). Stimulation of S1PR5 with A-971432, a selective agonist, preserves blood–brain barrier integrity and exerts therapeutic effect in an animal model of Huntington’s disease. Human Molecular Genetics, 27(14), 2490–2501.PubMedCrossRef
Ding, G., Sonoda, H., Yu, H., Kajimoto, T., Goparaju, S. K., Jahangeer, S., et al. (2007). Protein kinase D-mediated phosphorylation and nuclear export of sphingosine kinase 2. Journal of Biological Chemistry, 282(37), 27493–27502.CrossRefPubMed
Dinkins, M. B., Enasko, J., Hernandez, C., Wang, G., Kong, J., Helwa, I., et al. (2016). Neutral Sphingomyelinase-2 deficiency ameliorates Alzheimer’s disease pathology and improves cognition in the 5XFAD mouse. Journal of Neuroscience, 36(33), 8653–8667.PubMedCrossRef
Diop, F., Vial, T., Ferraris, P., Wichit, S., Bengue, M., Hamel, R., et al. (2018). Zika virus infection modulates the metabolomic profile of microglial cells. PLoS ONE, 13(10), e0206093.PubMedPubMedCentralCrossRef
Dodge, J. C., Clarke, J., Treleaven, C. M., Taksir, T. V., Griffiths, D. A., Yang, W., et al. (2009). Intracerebroventricular infusion of acid sphingomyelinase corrects CNS manifestations in a mouse model of Niemann-Pick A disease. Experimental Neurology, 215(2), 349–357.PubMedCrossRef
Dodge, J. C., Treleaven, C. M., Pacheco, J., Cooper, S., Bao, C., Abraham, M., et al. (2015). Glycosphingolipids are modulators of disease pathogenesis in amyotrophic lateral sclerosis. Proceedings of the National Academy of Sciences, 112(26), 8100–8105.CrossRef
Dohrn, M. F., Othman, A., Hirshman, S. K., Bode, H., Alecu, I., Fähndrich, E., et al. (2015). Elevation of plasma 1-deoxy-sphingolipids in type 2 diabetes mellitus: A susceptibility to neuropathy? European Journal of Neurology, 22(5), 806-e55.PubMedCrossRef
Dominguez, G., Maddelein, M. L., Pucelle, M., Nicaise, Y., Maurage, C. A., Duyckaerts, C., et al. (2018). Neuronal sphingosine kinase 2 subcellular localization is altered in Alzheimer’s disease brain. Acta Neuropathologica Communications, 6(1), 25.PubMedPubMedCentralCrossRef
Dworski, S., Lu, P., Khan, A., Maranda, B., Mitchell, J. J., Parini, R., et al. (2017). Acid ceramidase deficiency is characterized by a unique plasma cytokine and ceramide profile that is altered by therapy. Biochimica et Biophysica Acta (BBA)—Molecular Basis of Disease, 1863(2), 386–394.CrossRef
Eichler, F., & Van Haren, K. (2007). Immune response in leukodystrophies. Pediatric Neurology, 37(4), 235–244.PubMedCrossRef
Ernst, D., Murphy, S. M., Sathiyanadan, K., Wei, Y., Othman, A., Laurá, M., et al. (2015). Novel HSAN1 mutation in serine palmitoyltransferase resides at a putative phosphorylation site that is involved in regulating substrate specificity. Neuromolecular Medicine, 17(1), 47–57.PubMedPubMedCentralCrossRef
Farfel-Becker, T., Vitner, E. B., Kelly, S. L., Bame, J. R., Duan, J., Shinder, V., et al. (2014). Neuronal accumulation of glucosylceramide in a mouse model of neuronopathic Gaucher disease leads to neurodegeneration. Human Molecular Genetics, 23(4), 843–854.PubMedCrossRef
Farrell, D. F., & McKhann, G. M. (1971). Characterization of cerebroside sulfotransferase from rat brain. Journal of Biological Chemistry, 246(15), 4694–4702.CrossRefPubMed
Figuera-Losada, M., Stathis, M., Dorskind, J. M., Thomas, A. G., Bandaru, V. V. R., Yoo, S. W., et al. (2015). Cambinol, a novel inhibitor of neutral sphingomyelinase 2 shows neuroprotective properties. PLoS ONE, 10(5), 1–18.CrossRef
Fox, T. E., Houck, K. L., O’Neill, S. M., Nagarajan, M., Stover, T. C., Pomianowski, P. T., et al. (2007). Ceramide recruits and activates protein kinase C ζ (PKCζ) within structured membrane microdomains. Journal of Biological Chemistry, 282(17), 12450–12457.CrossRefPubMed
Frey, R. S., Rahman, A., Kefer, J. C., Minshall, R. D., & Malik, A. B. (2002). PKCζ regulates TNF-α–induced activation of NADPH oxidase in endothelial cells. Circulation Research, 90(9), 1012–1019.PubMedCrossRef
Furukawa, K., Ohmi, Y., Ji, S., Zhang, P., Bhuiyan, R. H., Ohkawa, Y., et al. (2017). Glycolipids: Essential regulator of neuro-inflammation, metabolism and gliomagenesis. Biochimica et Biophysica Acta BBA, 1861(10), 2479–2484.CrossRef
Gault, C. R., Obeid, L. M., & Hannun, Y. A. (2010). An overview of sphingolipid metabolism: From synthesis to breakdown. Sphingolipids as signaling and regulatory molecules. New York, NY: Springer.
Gill, J. S., & Windebank, A. J. (2000). Ceramide initiates NFκB-mediated caspase activation in neuronal apoptosis. Neurobiology of Disease, 7(4), 448–461.PubMedCrossRef
Giri, S., Khan, M., Rattan, R., Singh, I., & Singh, A. K. (2006). Krabbe disease: Psychosine-mediated activation of phospholipase A2 in oligodendrocyte cell death. Journal of Lipid Research, 47(7), 1478–1492.PubMedCrossRef
Gómez-Muñoz, A. (2006). Ceramide 1-phosphate/ceramide, a switch between life and death. Biochimica Biophysica Acta, 1758(12), 2049–2056.CrossRef
Grimm, M. O., Grimm, H. S., Pätzold, A. J., Zinser, E. G., Halonen, R., Duering, M., et al. (2005). Regulation of cholesterol and sphingomyelin metabolism by amyloid-beta and presenilin. Nature Cell Biology, 7(11), 1118–1123.PubMedCrossRef
Gustafson, S. J., Barth, B. M., McGill, C. M., Clausen, T. P., & Kuhn, T. B. (2007). Wild Alaskan blueberry extracts inhibit a magnesium-dependent sphingomyelinase activity in neurons exposed to TNFα. Current Topics in Nutraceutical Research, 5(4), 183–188.
Hagen, N., Van Veldhoven, P. P., Proia, R. L., Park, H., Merrill, A. H., & van Echten-Deckert, G. (2009). Subcellular origin of sphingosine 1-phosphate is essential for its toxic effect in lyase-deficient neurons. Journal of Biological Chemistry, 284(17), 11346–11353.CrossRefPubMedPubMedCentral
Hait, N. C., Allegood, J., Maceyka, M., Strub, G. M., Harikumar, K. B., Singh, S. K., et al. (2009). Regulation of histone acetylation in the nucleus by sphingosine-1 phosphate. Science, 325(5945), 1254–1257.PubMedPubMedCentralCrossRef
Hannun, Y. A., & Obeid, L. M. (2018). Sphingolipids and their metabolism in physiology and disease. Nature Reviews Molecular Cell Biology, 19(3), 175–191.PubMedCrossRef
Henriques, A., Croixmarie, V., Bouscary, A., Mosbach, A., Keime, C., Boursier-Neyret, C., et al. (2018). Sphingolipid metabolism is dysregulated at transcriptomic and metabolic levels in the spinal cord of an animal model of amyotrophic lateral sclerosis. Frontiers in Molecular Neuroscience, 10, 433.PubMedPubMedCentralCrossRef
Herr, D. R., Reolo, M. J., Peh, Y. X., Wang, W., Lee, C. W., Rivera, R., et al. (2016). Sphingosine-1-phosphate receptor 2 (S1P2) attenuates reactive oxygen species formation and inhibits cell death: Implications for otoprotective therapy. Scientific Reports, 6, 24541.PubMedPubMedCentralCrossRef
Hirabayashi, Y., Igarashi, Y., & Merrill, A. J. (2006). Sphingolipid biology. New York, NY: Springer Science & Business Media.CrossRef
Hou, L., Sun, F., Huang, R., Sun, W., Zhang, D., & Wang, Q. (2019). Inhibition of NADPH oxidase by apocynin prevents learning and memory deficits in a mouse Parkinson’s disease model. Redox Biology, 22, 101134.PubMedPubMedCentralCrossRef
Huang, Y., Li, Y., Zhang, H., Zhao, R., Jing, R., Xu, Y., et al. (2018). Zika virus propagation and release in human fetal astrocytes can be suppressed by neutral sphingomyelinase-2 inhibitor GW4869. Cell Discovery, 4(19), 1–16.
Ilyas, A. A., Chen, Z. W., & Cook, S. D. (2003). Antibodies to sulfatide in cerebrospinal fluid of patients with multiple sclerosis. Journal of Neuroimmunology, 139(1–2), 76–80.PubMedCrossRef
Imarisio, S., Carmichael, J., Korolchuk, V., Chen, C. W., Saiki, S., Rose, C., et al. (2008). Huntington’s disease: From pathology and genetics to potential therapies. Biochemical Journal, 412(2), 191–209.CrossRefPubMed
Jackman, N., Ishii, A., & Bansal, R. (2009). Oligodendrocyte development and myelin biogenesis: Parsing out the roles of glycosphingolipids. Physiology, 24(5), 290–297.PubMedCrossRef
Jana, A., & Pahan, K. (2004). Human immunodeficiency virus type 1 gp120 induces apoptosis in human primary neurons through redox-regulated activation of neutral sphingomyelinase. Journal of Neuroscience, 24(43), 9531–9540.PubMedCrossRef
Jana, M., Palencia, C. A., & Pahan, K. (2008). Fibrillar amyloid-β peptides activate microglia via TLR2: Implications for Alzheimer’s disease. The Journal of Immunology, 181(10), 7254–7262.PubMedCrossRef
Jazvinšćak Jembrek, M., Hof, P. R., & Šimić, G. (2015). Ceramides in Alzheimer’s disease: Key mediators of neuronal apoptosis induced by oxidative stress and Aβ accumulation. Oxidative Medicine and Cellular Longevity, 2015, 1–17.CrossRef
Jennemann, R., Sandhoff, R., Wang, S., Kiss, E., Gretz, N., Zuliani, C., et al. (2005). Cell-specific deletion of glucosylceramide synthase in brain leads to severe neural defects after birth. Proceedings of the National Academy of Sciences, 102(35), 12459–12464.CrossRef
Jeon, S. B., Yoon, H. J., Park, S. H., Kim, I. H., & Park, E. J. (2008). Sulfatide, a major lipid component of myelin sheath, activates inflammatory responses as an endogenous stimulator in brain-resident immune cells. The Journal of Immunology, 181(11), 8077–8087.PubMedCrossRef
Jeong, Y. H., Kim, Y., Song, H., Chung, Y. S., Park, S. B., & Kim, H. S. (2014). Anti-inflammatory effects of α-galactosylceramide analogs in activated microglia: Involvement of the p38 MAPK signaling pathway. PLoS ONE, 9(2), e87030.PubMedPubMedCentralCrossRef
Jęśko, H., Wencel, P. L., Wójtowicz, S., Strosznajder, J., Lukiw, W. J., & Strosznajder, R. P. (2020). Fingolimod affects transcription of genes encoding enzymes of ceramide metabolism in animal model of Alzheimer’s disease. Molecular Neurobiology, 57(6), 2799.PubMedPubMedCentralCrossRef
Jin, S., Zhou, F., Katirai, F., & Li, P. L. (2011). Lipid raft redox signaling: Molecular mechanisms in health and disease. Antioxidants & Redox Signaling, 15(4), 1043–1083.CrossRef
Johnson, K. R., Johnson, K. Y., Becker, K. P., Bielawski, J., Mao, C., & Obeid, L. M. (2003). Role of human sphingosine-1-phosphate phosphatase 1 in the regulation of intra-and extracellular sphingosine-1-phosphate levels and cell viability. Journal of Biological Chemistry, 278(36), 34541–34547.CrossRefPubMed
Jung, J. S., Shin, K. O., Lee, Y. M., Shin, J. A., Park, E. M., Jeong, J., et al. (2013). Anti-inflammatory mechanism of exogenous C2 ceramide in lipopolysaccharide-stimulated microglia. Biochimica et Biophysica Acta (BBA)—Molecular and Cell Biology of Lipids, 1831(6), 1016–1026.
Karaca, I., Tamboli, I. Y., Glebov, K., Richter, J., Fell, L. H., Grimm, M. O., et al. (2014). Deficiency of sphingosine-1-phosphate lyase impairs lysosomal metabolism of the amyloid precursor protein. Journal of Biological Chemistry, 289(24), 16761–16772.CrossRefPubMedPubMedCentral
Kataoka, H., Sugahara, K., Shimano, K., Teshima, K., Koyama, M., Fukunari, A., & Chiba, K. (2005). FTY720, sphingosine 1-phosphate receptor modulator, ameliorates experimental autoimmune encephalomyelitis by inhibition of T cell infiltration. Cellular & Molecular Immunology, 2(6), 439–448.
Kikumoto, Y., Kai, Y., Morinaga, H., Iga-Murahashi, M., Matsuyama, M., Sasaki, T., et al. (2010). Fabry disease exhibiting recurrent stroke and persistent inflammation. Internal Medicine, 49(20), 2247–2252.PubMedCrossRef
Kim, O. S., Park, E. J., Joe, E. H., & Jou, I. (2002). JAK-STAT signaling mediates gangliosides-induced inflammatory responses in brain microglial cells. Journal of Biological Chemistry, 277(43), 40594–40601.CrossRefPubMed
Kim, S., & Sieburth, D. (2018). Sphingosine kinase activates the mitochondrial unfolded protein response and is targeted to mitochondria by stress. Cell Reports, 24(11), 2932-2945.e4.PubMedCrossRef
Kitatani, K., Idkowiak-Baldys, J., & Hannun, Y. A. (2008). The sphingolipid salvage pathway in ceramide metabolism and signaling. Cellular Signaling, 20(6), 1010–1018.CrossRef
Kleinschnitz, C., Grund, H., Wingler, K., Armitage, M. E., Jones, E., Mittal, M., et al. (2010). Post-stroke inhibition of induced NADPH oxidase type 4 prevents oxidative stress and neurodegeneration. PLoS Biology, 8(9), e1000479.PubMedPubMedCentralCrossRef
Kolter, T., Proia, R. L., & Sandhoff, K. (2002). Combinatorial ganglioside biosynthesis. Journal of Biological Chemistry, 277(29), 25859–25862.CrossRefPubMed
Kramer, R., Bielawski, J., Kistner-Griffin, E., Othman, A., Alecu, I., Ernst, D., et al. (2015). Neurotoxic 1-deoxysphingolipids and paclitaxel-induced peripheral neuropathy. FASEB Journal, 29(11), 4461–4472.PubMedPubMedCentralCrossRef
Kuhle, J., Lindberg, R. L. P., Regeniter, A., Mehling, M., Steck, A. J., Kappos, L., & Czaplinski, A. (2009). Increased levels of inflammatory chemokines in amyotrophic lateral sclerosis. European Journal of Neurology, 16(6), 771–774.PubMedCrossRef
Kułakowska, A., Żendzian-Piotrowska, M., Baranowski, M., Konończuk, T., Drozdowski, W., Górski, J., & Bucki, R. (2010). Intrathecal increase of sphingosine 1-phosphate at early stage multiple sclerosis. Neuroscience Letters, 477(3), 149–152.PubMedCrossRef
Lamour, N. F., Wijesinghe, D. S., Mietla, J. A., Ward, K. E., Stahelin, R. V., & Chalfant, C. E. (2011). Ceramide kinase regulates the production of tumor necrosis factor α (TNFα) via inhibition of TNFα-converting enzyme. Journal of Biological Chemistry, 286(50), 42808–42817.CrossRefPubMedPubMedCentral
Lee, K. D., Chow, W. N., Sato-Bigbee, C., Graf, M. R., Graham, R. S., Colello, R. J., et al. (2009). FTY720 reduces inflammation and promotes functional recovery after spinal cord injury. Journal of Neurotrauma, 26(12), 2335–2344.PubMedPubMedCentralCrossRef
Lee, W. C., Tsoi, Y. K., Troendle, F. J., DeLucia, M. W., Ahmed, Z., Dicky, C. A., et al. (2007). Single-dose intracerebroventricular administration of galactocerebrosidase improves survival in a mouse model of globoid cell leukodystrophy. The FASEB Journal, 21(10), 2520–2527.PubMedCrossRef
Lei, M., Teo, J. D., Song, H., McEwen, H. P., Lee, J. Y., Couttas, T. A., et al. (2019). Sphingosine kinase 2 potentiates amyloid deposition but protects against hippocampal volume loss and demyelination in a mouse model of Alzheimer’s disease. Journal of Neuroscience, 39(48), 9645–9659.PubMedCrossRef
Leto, T. L., Morand, S., Hurt, D., & Ueyama, T. (2009). Targeting and regulation of reactive oxygen species generation by Nox family NADPH oxidases. Antioxidants & Redox Signaling, 11(10), 2607–2619.CrossRef
Levade, T., Moser, H. W., Fensom, A. H., Harzer, K., Moser, A. B., & Salvayre, R. (1995). Neurodegenerative course in ceramidase deficiency (Farber disease) correlates with the residual lysosomal ceramide turnover in cultured living patient cells. Journal of the Neurological Sciences, 134(1–2), 108–114.PubMedCrossRef
Li, X., Jin, S. J., Su, J., Li, X. X., & Xu, M. (2018). Acid sphingomyelinase down-regulation alleviates vascular endothelial insulin resistance in diabetic rats. Basic & Clinical Pharmacology & Toxicology, 123(6), 645–659.CrossRef
Li, Z., Hailemariam, T. K., Zhou, H., Li, Y., Duckworth, D. C., Peake, D. A., et al. (2007). Inhibition of sphingomyelin synthase (SMS) affects intracellular sphingomyelin accumulation and plasma membrane lipid organization. Biochimica et Biophysica Acta (BBA)—Molecular and Cell Biology of Lipids, 1771(9), 1186–1194.
Lloyd-Evans, E., Morgan, A. J., He, X., Smith, D. A., Elliot-Smith, E., Sillence, D. J., et al. (2008). Niemann-Pick disease type C1 is a sphingosine storage disease that causes deregulation of lysosomal calcium. Nature Medicine, 14(11), 1247.PubMedCrossRef
Lone, M. A., Santos, S., Alecu, I., Silva, L. C., & Hornemann, T. (2019). 1-Deoxysphingolipids. Biochimica et Biophysica Acta (BBA)—Molecular and Cell Biology of Lipids, 1864(4), 512–521.
Lugrin, J., Ciarlo, E., Santos, A., Grandmaison, G., Dos Santos, I., Le Roy, D., & Roger, T. (2013). The sirtuin inhibitor cambinol impairs MAPK signaling, inhibits inflammatory and innate immune responses and protects from septic shock. Biochimica et Biophysica Acta (BBA)—Molecular Cell Research, 1833(6), 1498–1510.CrossRef
Maceyka, M., Harikumar, K. B., Milstien, S., & Spiegel, S. (2012). Sphingosine-1-phosphate signaling and its role in disease. Trends in Cell Biology, 22(1), 50–60.PubMedCrossRef
Maceyka, M., Sankala, H., Hait, N. C., Le Stunff, H., Liu, H., Toman, R., et al. (2005). SphK1 and SphK2, sphingosine kinase isoenzymes with opposing functions in sphingolipid metabolism. Journal of Biological Chemistry, 280(44), 37118–37129.CrossRefPubMed
Maglione, V., Marchi, P., Di Pardo, A., Lingrell, S., Horkey, M., Tidmarsh, E., & Sipione, S. (2010). Impaired ganglioside metabolism in Huntington’s disease and neuroprotective role of GM1. Journal of Neuroscience, 30(11), 4072–4080.PubMedCrossRef
Marshall, J., Sun, Y., Bangari, D. S., Budman, E., Park, H., Nietupski, J. B., et al. (2016). CNS-accessible inhibitor of glucosylceramide synthase for substrate reduction therapy of neuronopathic Gaucher disease. Molecular Therapy, 24(6), 1019–1029.PubMedPubMedCentralCrossRef
Marshall, M. S., Jakubauskas, B., Bogue, W., Stoskute, M., Hauck, Z., Rue, E., et al. (2018). Analysis of age-related changes in psychosine metabolism in the human brain. PLoS ONE, 13(2), e0193438.PubMedPubMedCentralCrossRef
McCampbell, A., Truong, D., Broom, D. C., Allchorne, A., Gable, K., Cutler, R. G., et al. (2005). Mutant SPTLC1 dominantly inhibits serine palmitoyltransferase activity in vivo and confers as age-dependent neuropathy. Human Molecular Genetics, 14(22), 3507–3521.PubMedCrossRef
Miguez, A., Garcia-Diaz Barriga, G., Brito, V., Straccia, M., Giralt, A., Ginés, S., et al. (2015). Fingolimod (FTY720) enhances hippocampal synaptic plasticity and memory in Huntington’s disease by preventing p75NTR up-regulation and astrocyte-mediated inflammation. Human Molecular Genetics, 24(17), 4958–4970.PubMedCrossRef
Mitrofanova, A., Mallela, S. K., Ducasa, G. M., Yoo, T. H., Rosenfeld-Gur, E., Zelnik, I. D., et al. (2019). SMPDL3b modulates insulin receptor signaling in diabetic kidney disease. Nature Communications, 10(1), 2692.PubMedPubMedCentralCrossRef
Mitsutake, S., Date, T., Yokota, H., Sugiura, M., Kohama, T., & Igarashi, Y. (2012). Ceramide kinase deficiency improves diet-induced obesity and insulin resistance. FEBS Letters, 586(9), 1300–1305.PubMedCrossRef
Myerowitz, R. (1997). Tay-Sachs disease-causing mutations and neutral polymorphisms in the Hex A gene. Human Mutation, 9(3), 195–208.PubMedCrossRef
Neubauer, H. A., Tea, M. N., Zebol, J. R., Gliddon, B. L., Stefanidis, C., Moretti, P. A. B., et al. (2019). Cytoplasmic dynein regulates the subcellular localization of sphingosine kinase 2 to elicit tumor-suppressive functions in glioblastoma. Oncogene, 38(8), 1151–1165.PubMedCrossRef
Neumann, J., Bras, J., Deas, E., O’Sullivan, S. S., Parkkinen, L., Lachmann, R. H., et al. (2009). Glucocerebrosidase mutations in clinical and pathologically proven Parkinson’s disease. Brain, 132(7), 1783–1794.PubMedPubMedCentralCrossRef
Newton, J., Hait, N. C., Maceyka, M., Colaco, A., Maczis, M., Wassif, C. A., et al. (2017). FTY720/fingolimod increases NPC1 and NPC2 expression and reduces cholesterol and sphingolipid accumulation in Niemann-Pick type C mutant fibroblasts. The FASEB Journal, 31(4), 1719–1730.PubMedPubMedCentralCrossRef
Newton, J., Milstien, S., & Spiegel, S. (2018). Niemann-Pick type C disease: The atypical sphingolipidosis. Advances in Biological Regulation, 70, 82–88.PubMedPubMedCentralCrossRef
Norman, E., Cutler, R. G., Flannery, R., Wang, Y., & Mattson, M. P. (2010). Plasma membrane sphingomyelin hydrolysis increases hippocampal neuron excitability by sphingosine-1-phosphate mediated mechanisms. Journal of Neurochemistry, 114(2), 430–439.PubMedPubMedCentralCrossRef
Othman, A., Bianchi, R., Alecu, I., Wei, Y., Porretta-Serapiglia, C., Lombardi, R., et al. (2015). Lowering plasma 1-deoxysphingolipids improves neuropathy in diabetic rats. Diabetes, 64(3), 1035–1045.PubMedCrossRef
Paciotti, S., Albi, E., Parnetti, L., & Beccari, T. (2020). Lysosomal ceramide metabolism disorders: Implications in Parkinson’s disease. Journal of Clinical Medicine, 9(2), 594.PubMedCentralCrossRef
Pasqui, A. L., Di Renzo, M., Auteri, A., Federico, G., & Puccetti, L. (2007). Increased TNF-α production by peripheral blood mononuclear cells in patients with Krabbe’s disease: Effect of psychosine. European Journal of Clinical Investigation, 37(9), 742–745.PubMedCrossRef
Penno, A., Reilly, M. M., Houlden, H., Laurá, M., Rentsch, K., Niederkofler, V., et al. (2010). Hereditary sensory neuropathy type 1 is caused by the accumulation of two neurotoxic sphingolipids. Journal of Biological Chemistry, 285(15), 11178–11187.CrossRefPubMedPubMedCentral
Persidsky, Y., Buttini, M., Limoges, J., Bock, P., & Gendelman, H. E. (1997). An analysis of HIV-1-associated inflammatory products in brain tissue of humans and SCID mice with HIV-1 encephalitis. Journal of Neurovirology, 3(6), 401–416.PubMedCrossRef
Pettus, B. J., Bielawska, A., Subramanian, P., Wijesinghe, D. S., Maceyka, M., Leslie, C. C., et al. (2004). Ceramide 1-phosphate is a direct activator of cytosolic phospholipase A2. Journal of Biological Chemistry, 279(12), 11320–11326.CrossRefPubMed
Porubsky, S., Jennemann, R., Lehmann, L., & Gröne, H. J. (2014). Depletion of globosides and isoglobosides fully reverts the morphologic phenotype of Fabry disease. Cell and Tissue Research, 358(1), 217–227.PubMedPubMedCentralCrossRef
Potenza, R. L., De Simone, R., Armida, M., Mazziotti, V., Pèzzola, A., Popoli, P., & Minghetti, L. (2016). Fingolimod: A disease-modifier drug in a mouse model of amyotrophic lateral sclerosis. Neurotherapeutics, 13(4), 918–927.PubMedPubMedCentralCrossRef
Robak, L. A., Jansen, I. E., Van Rooij, J., Uitterlinden, A. G., Kraaij, R., Jankovic, J., et al. (2017). Excessive burden of lysosomal storage disorder gene variants in Parkinson’s disease. Brain, 140(12), 3191–3203.PubMedPubMedCentralCrossRef
Rombach, S. M., Smid, B. E., Bouwman, M. G., Linthorst, G. E., Dijkgraaf, M. G., & Hollak, C. E. (2013). Long term enzyme replacement therapy for Fabry disease: Effectiveness on kidney, heart and brain. Orphanet Journal of Rare Diseases, 8(1), 47.PubMedPubMedCentralCrossRef
Rothhammer, V., Kenison, J. E., Tjon, E., Takenaka, M. C., de Lima, K. A., Borucki, D. M., et al. (2017). Sphingosine 1-phosphate receptor modulation suppresses pathogenic astrocyte activation and chronic progressive CNS inflammation. Proceedings of the National Academy of Sciences, 114(8), 2012–2017.CrossRef
Schiffmann, R., FitzGibbon, E. J., Harris, C., DeVile, C., Davies, E. H., Abel, L., et al. (2008). Randomized, controlled trial of miglustat in Gaucher’s disease type 3. Annals of Neurology: Official Journal of the American Neurological Association and the Child Neurology Society, 64(5), 514–522.CrossRef
Schlotawa, L., Adang, L. A., Radhakrishnan, K., & Ahrens-Nicklas, R. C. (2020). Multiple sulfatase deficiency: A disease comprising mucopolysaccharidosis, sphingolipidosis, and more caused by a defect in posttranslational modification. International Journal of Molecular Sciences, 21(10), 3448.PubMedCentralCrossRef
Schuchman, E. H., & Desnick, R. J. (2017). Types A and B Niemann-pick disease. Molecular Genetics and Metabolism, 120(1–2), 27–33.PubMedCrossRef
Sechi, A., Deroma, L., Dardis, A., Ciana, G., Bertin, N., Concolino, D., et al. (2014). Long term effects of enzyme replacement therapy in an Italian cohort of type 3 Gaucher patients. Molecular Genetics and Metabolism, 113(3), 213–218.PubMedCrossRef
Senkal, C. E., Salama, M. F., Snider, A. J., Allopenna, J. J., Rana, N. A., Koller, A., et al. (2017). Ceramide is metabolized to acylceramide and stored in lipid droplets. Cell Metabolism, 25(3), 686–697.PubMedPubMedCentralCrossRef
Settembre, C., Annunziata, I., Spampanato, C., Zarcone, D., Cobellis, G., Nusco, E., et al. (2007). Systemic inflammation and neurodegeneration in a mouse model of multiple sulfatase deficiency. Proceedings of the National Academy of Sciences, 104(11), 4506–4511.CrossRef
Seyfried, T. N., Choi, H., Chevalier, A., Hogan, D., Akgoc, Z., & Schneider, J. S. (2018). Sex-related abnormalities in substantia nigra lipids in Parkinson’s disease. ASN Neuro, 10, 1–10.CrossRef
Seyrantepe, V., Demir, S. A., Timur, Z. K., Von Gerichten, J., Marsching, C., Erdemli, E., et al. (2018). Murine Sialidase Neu3 facilitates GM2 degradation and bypass in mouse model of Tay-Sachs disease. Experimental Neurology, 299, 26–41.PubMedCrossRef
Shanbhogue, P., & Hannun, Y. A. (2020). Exploring the therapeutic landscape of sphingomyelinases. Handbook of Experimental Pharmacology, 259, 19–47.PubMedCrossRef
Shinghal, R., Scheller, R. H., & Bajjalieh, S. M. (1993). Ceramide-1-phosphate phosphatase activity in brain. Journal of Neurochemistry, 61(6), 2279–2285.PubMedCrossRef
Shukitt-Hale, B., Thangthaeng, N., Miller, M. G., Poulose, S. M., Carey, A. N., & Fisher, D. R. (2019). Blueberries improve neuroinflammation and cognition differentially depending on individual cognitive baseline status. The Journals of Gerontology: Series A, 74(7), 977–983.
Siow, D. L., Anderson, C. D., Berdyshev, E. V., Skobeleva, A., Natarajan, V., Pitson, S. M., et al. (2011). Sphingosine kinase localization in the control of sphingolipid metabolism. Advances in Enzyme Regulation, 51(1), 229–244.PubMedCrossRef
Siow, D. L., Anderson, C. D., Berdyshev, E. V., Skobeleva, A., Pitson, S. M., & Wattenberg, B. W. (2010). Intracellular localization of sphingosine kinase 1 alters access to substrate pools but does not affect the degradative fate of sphingosine-1-phosphate. Journal of Lipid Research, 51(9), 2546–2559.PubMedPubMedCentralCrossRef
Sivasubramanian, M., Kanagaraj, N., Dheen, S. T., & Tay, S. S. W. (2015). Sphingosine kinase 2 and sphingosine-1-phosphate promotes mitochondrial function in dopaminergic neurons of mouse model of Parkinson’s disease and in MPP+-treated MN9D cells in vitro. Neuroscience, 290, 636–648.PubMedCrossRef
Smith, D., Wallom, K. L., Williams, I. M., Jeyakumar, M., & Platt, F. M. (2009). Beneficial effects of anti-inflammatory therapy in a mouse model of Niemann-Pick disease type C1. Neurobiology of Disease, 36(2), 242–251.PubMedCrossRef
Suriyanarayanan, S., Auranen, M., Toppila, J., Paetau, A., Shcherbii, M., Palin, E., et al. (2016). The Variant p.(Arg183Trp) in SPTLC2 causes late-onset hereditary sensory neuropathy. Neuromolecular Medicine, 18(1), 81–90.PubMedCrossRef
Tafesse, F. G., Vacaru, A. M., Bosma, E. F., Hermansson, M., Jain, A., Hilderink, A., et al. (2014). Sphingomyelin synthase-related protein SMSr is a suppressor of ceramide-induced mitochondrial apoptosis. Journal of Cell Science, 127(2), 445–454.PubMed
Takajo, D., Matsumoto, H., Noguchi, T., Nishimura, N., & Nonoyama, S. (2020). New variant mutation of glucosylceramidase beta (GBA) and early enzyme replacement therapy for neuronopathic gaucher disease: A case report and literature review. Iranian Journal of Pediatrics. https://​doi.​org/​10.​5812/​ijp.​98996.CrossRef
Takasugi, N., Sasaki, T., Suzuki, K., Osawa, S., Isshiki, H., Hori, Y., et al. (2011). BACE1 activity is modulated by cell-associated sphingosine-1-phosphate. Journal of Neuroscience, 31(18), 6850–6857.PubMedCrossRef
Tappino, B., Biancheri, R., Mort, M., Regis, S., Corsolini, F., Rossi, A., et al. (2010). Identification and characterization of 15 novel GALC gene mutations causing Krabbe disease. Human Mutation, 31(12), E1894–E1914.PubMedPubMedCentralCrossRef
Trajkovic, K., Hsu, C., Chiantia, S., Rajendran, L., Wenzel, D., Wieland, F., et al. (2008). Ceramide triggers budding of exosome vesicles into multivesicular endosomes. Science, 319(5867), 1244–1247.PubMedCrossRef
Tsai, H. C., & Han, M. H. (2016). Sphingosine-1-phosphate (S1P) and S1P signaling pathway: therapeutic targets in autoimmunity and inflammation. Drugs, 76(11), 1067–1079.PubMedCrossRef
Uemura, S., Go, S., Shishido, F., & Inokuchi, J. I. (2014). Expression machinery of GM4: The excess amounts of GM3/GM4S synthase (ST3GAL5) are necessary for GM4 synthesis in mammalian cells. Glycoconjugate Journal, 31(2), 101–108.PubMedCrossRef
Vacaru, A. M., Tafesse, F. G., Ternes, P., Kondylis, V., Hermansson, M., Brouwers, J. F., et al. (2009). Sphingomyelin synthase-related protein SMSr controls ceramide homeostasis in the ER. Journal of Cell Biology, 185(6), 1013–1027.CrossRefPubMedPubMedCentral
Van Doorn, R., Van Horssen, J., Verzijl, D., Witte, M., Ronken, E., Van Het Hof, B., et al. (2010). Sphingosine 1-phosphate receptor 1 and 3 are upregulated in multiple sclerosis lesions. Glia, 58(12), 1465–1476.PubMedCrossRef
Venkatesan, A., & Benavides, D. R. (2015). Autoimmune encephalitis and its relation to infection. Current Neurology and Neuroscience Reports, 15(3), 1–11.CrossRef
Villani, M., Subathra, M., Im, Y. B., Choi, Y., Signorelli, P., Del Poeta, M., & Luberto, C. (2008). Sphingomyelin synthases regulate production of diacylglycerol at the Golgi. Biochemical Journal, 414(1), 31–41.CrossRefPubMed
Vitner, E. B., Farfel-Becker, T., Eilam, R., Biton, I., & Futerman, A. H. (2012). Contribution of brain inflammation to neuronal cell death in neuronopathic forms of Gaucher’s disease. Brain, 135(6), 1724–1735.PubMedCrossRef
Wang, J., Liu, J., Zhou, R., Ding, X., Zhang, Q., Zhang, C., et al. (2018). Zika virus infected primary microglia impairs NPCs proliferation and differentiation. Biochemical and Biophysical Research Communications, 497(2), 619–625.PubMedCrossRef
Wang, Q., Qian, L., Chen, S. H., Chu, C. H., Wilson, B., Oyarzabal, E., et al. (2015). Post-treatment with an ultra-low dose of NADPH oxidase inhibitor diphenyleneiodonium attenuates disease progression in multiple Parkinson’s disease models. Brain, 138(5), 1247–1262.PubMedPubMedCentralCrossRef
Wang, W., Shanmugam, M. K., Xiang, P., Yam, T. Y. A., Kumar, V., Chew, W. S., et al. (2020a). Sphingosine-1-phosphate receptor 2 induces otoprotective responses to cisplatin treatment. Cancers, 12(1), 211.PubMedCentralCrossRef
Weiner, H. L. (2004). Multiple sclerosis is an inflammatory T-cell–mediated autoimmune disease. Archives of Neurology, 61(10), 1613–1615.PubMedCrossRef
White, A. J., Wijeyekoon, R. S., Scott, K. M., Gunawardana, N. P., Hayat, S., Solim, I. H., et al. (2018). The peripheral inflammatory response to alpha-synuclein and endotoxin in Parkinson’s disease. Frontiers in Neurology, 9, 946.PubMedPubMedCentralCrossRef
Wilson, E. R., Kugathasan, U., Abramov, A. Y., Clark, A. J., Bennett, D. L. H., Reilly, M. M., et al. (2018). Hereditary sensory neuropathy type 1-associated deoxysphingolipids cause neurotoxicity, acute calcium handling abnormalities and mitochondrial dysfunction in vitro. Neurobiology of Disease, 117, 1–14.PubMedPubMedCentralCrossRef
Wong, Y. C., & Krainc, D. (2016). Lysosomal trafficking defects link Parkinson’s disease with Gaucher’s disease. Movement Disorders, 31(11), 1610–1618.PubMedPubMedCentralCrossRef
Wu, D. C., Ré, D. B., Nagai, M., Ischiropoulos, H., & Przedborski, S. (2006). The inflammatory NADPH oxidase enzyme modulates motor neuron degeneration in amyotrophic lateral sclerosis mice. Proceedings of the National Academy of Sciences, 103(32), 12132–12137.CrossRef
Wu, G., Yan, B., Wang, X., Feng, X., Zhang, A., Xu, X., & Dong, H. (2008). Decreased activities of lysosomal acid alpha-D-galactosidase A in the leukocytes of sporadic Parkinson’s disease. Journal of the Neurological Sciences, 271(1–2), 168–173.PubMedCrossRef
Wu, Y. P., Mizukami, H., Matsuda, J., Saito, Y., Proia, R. L., & Suzuki, K. (2005). Apoptosis accompanied by up-regulation of TNF-α death pathway genes in the brain of Niemann-Pick type C disease. Molecular Genetics and Metabolism, 84(1), 9–17.PubMedCrossRef
Young, M. M., Takahashi, Y., Fox, T. E., Yun, J. K., Kester, M., & Wang, H. G. (2016). Sphingosine kinase 1 cooperates with autophagy to maintain endocytic membrane trafficking. Cell Reports, 17(6), 1532–1545.PubMedCrossRef
Zhou, W., Woodson, M., Sherman, M. B., Neelakanta, G., & Sultana, H. (2019). Exosomes mediate Zika virus transmission through SMPD3 neutral sphingomyelinase in cortical neurons. Emerging Microbes & Infections, 8(1), 307–326.CrossRef
Zunke, F., Moise, A. C., Belur, N. R., Gelyana, E., Stojkovska, I., Dzaferbegovic, H., et al. (2018). Reversible conformational conversion of α-synuclein into toxic assemblies by glucosylceramide. Neuron, 97(1), 92–107.PubMedCrossRef
Metadata
Title
Sphingolipids as Regulators of Neuro-Inflammation and NADPH Oxidase 2
Authors
Emma J. Arsenault
Colin M. McGill
Brian M. Barth
Publication date
01-03-2021
Publisher
Springer US
Published in
NeuroMolecular Medicine / Issue 1/2021
Print ISSN: 1535-1084
Electronic ISSN: 1559-1174
DOI
https://doi.org/10.1007/s12017-021-08646-2

Other articles of this Issue 1/2021

NeuroMolecular Medicine 1/2021 Go to the issue

Review Paper

Lysophosphatidic Acid Signalling in Nervous System Development and Function

Review Paper

Recent Insights on the Role of PPAR-β/δ in Neuroinflammation and Neurodegeneration, and Its Potential Target for Therapy

S.I : Translational aspects of Lipid Biology in the Brain

Docosahexaenoic Acid (DHA) Supplementation Alters Phospholipid Species and Lipid Peroxidation Products in Adult Mouse Brain, Heart, and Plasma

Review Paper

Preclinical and Clinical Evidence for the Involvement of Sphingosine 1-Phosphate Signaling in the Pathophysiology of Vascular Cognitive Impairment

Review Paper

Bile Acids: A Communication Channel in the Gut-Brain Axis

Original Paper

A Walnut Diet in Combination with Enriched Environment Improves Cognitive Function and Affects Lipid Metabolites in Brain and Liver of Aged NMRI Mice