Sphingolipid Abnormalities in Encephalomyeloradiculoneuropathy (EMRN) Are Associated with an Anti-neutral Glycolipid Antibody

Overview

Journal FEBS Open Bio

Specialty Biology

Date 2023 Feb 22

PMID 36807737

Authors

Tatsuro Mutoh

Akihiro Ueda

Yoshiki Niimi

Affiliations

Soon will be listed here.

Abstract

Accumulating evidence suggests that various sphingolipids and glycosphingolipids can act as mediators for inflammation or signaling molecules in the nervous system. In this article, we explore the molecular basis of a new neuroinflammatory disorder called encephalomyeloradiculoneuropathy (EMRN), which affects the brain, spinal cord, and peripheral nerves; in particular, we discuss whether glycolipid and sphingolipid dysmetabolism is present in patients with this disorder. This review will focus on the pathognomonic significance of sphingolipid and glycolipid dysmetabolism for the development of EMRN and the possible involvement of inflammation in the nervous system.

Citing Articles

Combined central and peripheral demyelination: a case report resembling encephalomyeloradiculoneuropathy.

Zhou X, Peng A, Li C, Li L, Yao D, Hao Y Front Neurol. 2024; 14:1288546.

PMID: 38292033 PMC: 10825037. DOI: 10.3389/fneur.2023.1288546.

An open chat with… Sandro Sonnino.

Tsagakis I, Sonnino S FEBS Open Bio. 2023; 13(9):1544-1547.

PMID: 37589211 PMC: 10476558. DOI: 10.1002/2211-5463.13689.

References

Vitner E, Farfel-Becker T, Eilam R, Biton I, Futerman A . Contribution of brain inflammation to neuronal cell death in neuronopathic forms of Gaucher's disease. Brain. 2012; 135(Pt 6):1724-35. DOI: 10.1093/brain/aws095. View

Kinoshita A, Hayashi M, Miyamoto K, Oda M, Tanabe H . Inflammatory demyelinating polyradiculitis in a patient with acute disseminated encephalomyelitis (ADEM). J Neurol Neurosurg Psychiatry. 1996; 60(1):87-90. PMC: 486196. DOI: 10.1136/jnnp.60.1.87. View

Sidransky E, Nalls M, Aasly J, Aharon-Peretz J, Annesi G, Barbosa E . Multicenter analysis of glucocerebrosidase mutations in Parkinson's disease. N Engl J Med. 2009; 361(17):1651-61. PMC: 2856322. DOI: 10.1056/NEJMoa0901281. View

Pujol-Lereis L . Alteration of Sphingolipids in Biofluids: Implications for Neurodegenerative Diseases. Int J Mol Sci. 2019; 20(14). PMC: 6678458. DOI: 10.3390/ijms20143564. View

Rotolo J, Stancevic B, Zhang J, Hua G, Fuller J, Yin X . Anti-ceramide antibody prevents the radiation gastrointestinal syndrome in mice. J Clin Invest. 2012; 122(5):1786-90. PMC: 3336980. DOI: 10.1172/JCI59920. View

Krishnamurthy K, Dasgupta S, Bieberich E . Development and characterization of a novel anti-ceramide antibody. J Lipid Res. 2007; 48(4):968-75. DOI: 10.1194/jlr.D600043-JLR200. View

Norton W, AUTILIO L . The lipid composition of purified bovine brain myelin. J Neurochem. 1966; 13(4):213-22. DOI: 10.1111/j.1471-4159.1966.tb06794.x. View

Pannu R, Won J, Khan M, Singh A, Singh I . A novel role of lactosylceramide in the regulation of lipopolysaccharide/interferon-gamma-mediated inducible nitric oxide synthase gene expression: implications for neuroinflammatory diseases. J Neurosci. 2004; 24(26):5942-54. PMC: 6729229. DOI: 10.1523/JNEUROSCI.1271-04.2004. View

Shima S, Kawamura N, Ishikawa T, Masuda H, Iwahara C, Niimi Y . Anti-neutral glycolipid antibodies in encephalomyeloradiculoneuropathy. Neurology. 2013; 82(2):114-8. DOI: 10.1212/WNL.0000000000000015. View

10.

Mutoh T, Niimi Y, Ito S, Akiyama H, Shiroki R, Hirabayashi Y . A pilot study assessing sphingolipids and glycolipids dysmetabolism in idiopathic normal pressure hydrocephalus. Biochem Biophys Res Commun. 2022; 639:84-90. DOI: 10.1016/j.bbrc.2022.11.091. View

11.

Maceyka M, Spiegel S . Sphingolipid metabolites in inflammatory disease. Nature. 2014; 510(7503):58-67. PMC: 4320971. DOI: 10.1038/nature13475. View

12.

Catani M, Gasperi V, Bisogno T, Maccarrone M . Essential Dietary Bioactive Lipids in Neuroinflammatory Diseases. Antioxid Redox Signal. 2017; 29(1):37-60. PMC: 5984567. DOI: 10.1089/ars.2016.6958. View

13.

Lee J, Kim J, Park S, Sim Y, Jung J, Suh H . Lactosylceramide Mediates the Expression of Adhesion Molecules in TNF-α and IFNγ-stimulated Primary Cultured Astrocytes. Korean J Physiol Pharmacol. 2011; 15(5):251-8. PMC: 3222793. DOI: 10.4196/kjpp.2011.15.5.251. View

14.

Ueda A, Shima S, Murate K, Kikuchi K, Nagao R, Maeda T . Anti-GM1 ganglioside antibodies modulate membrane-associated sphingomyelin metabolism by altering neutral sphingomyelinase activity. Mol Cell Neurosci. 2018; 89:42-48. DOI: 10.1016/j.mcn.2018.03.012. View

15.

Mihara T, Ueda A, Hirayama M, Takeuchi T, Yoshida S, Naito K . Detection of new anti-neutral glycosphingolipids antibodies and their effects on Trk neurotrophin receptors. FEBS Lett. 2006; 580(21):4991-5. DOI: 10.1016/j.febslet.2006.08.013. View

16.

Mencarelli C, Martinez-Martinez P . Ceramide function in the brain: when a slight tilt is enough. Cell Mol Life Sci. 2012; 70(2):181-203. PMC: 3535405. DOI: 10.1007/s00018-012-1038-x. View

17.

Kota V, Hama H . 2'-Hydroxy ceramide in membrane homeostasis and cell signaling. Adv Biol Regul. 2013; 54:223-30. PMC: 3946364. DOI: 10.1016/j.jbior.2013.09.012. View

18.

OBrien J, Sampson E . Lipid composition of the normal human brain: gray matter, white matter, and myelin. J Lipid Res. 1965; 6(4):537-44. View

19.

Schumacher F, Edwards M, Muhle C, Carpinteiro A, Wilson G, Wilker B . Ceramide levels in blood plasma correlate with major depressive disorder severity and its neutralization abrogates depressive behavior in mice. J Biol Chem. 2022; 298(8):102185. PMC: 9304786. DOI: 10.1016/j.jbc.2022.102185. View

20.

Yamaguchi A, Katsuyama K, Nagahama K, Takai T, Aoki I, Yamanaka S . Possible role of autoantibodies in the pathophysiology of GM2 gangliosidoses. J Clin Invest. 2004; 113(2):200-8. PMC: 311432. DOI: 10.1172/JCI19639. View