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Changes in the Metabolism of Sphingomyelin and Ceramide in the Brain Structures and Spinal Cord of Transgenic Mice (FUS(1-359)) Modeling Amyotrophic Lateral Sclerosis

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Abstract

Changes in the expression of genes for enzymes of sphingolipid metabolism in transgenic FUS mice (FUS (1-359)) simulating amyotrophic lateral sclerosis (ALS) were found. The profiles of molecular species of sphingomyelins (SM) and ceramides (CER) in the structure of the brain and in the spinal cord were analyzed. During the development of ALS, changes in the spectrum of molecular types of CER were noted only in the spinal cord, where almost all CER decreased in relation to the control after two and three months of the course of the disease. After four months of the development of the pathology, an increase in the expression of the acid ceramidase gene was observed, as a result of which sphingosine can be formed, which has pronounced proapoptotic properties. Analysis of gene expression of CER and galactosylceramide (GalCER) metabolism enzymes indicates the possibility of GalCER functioning as a source for maintaining CER levels at the terminal stage of ALS. Thus, the metabolism of sphingolipids is a promising field of study of the processes associated with ALS, both in the context of the search for potential diagnostic markers and effective drugs, and for explaining the pathogenesis of ALS.

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ACKNOWLEDGMENTS

Maintenance of the animals was provided by the Program for Support of Bioresource Collections of the Institute of Physiologically Active Substances of the Russian Academy of Sciences and carried out on the equipment of the Center for Collective Use of the Institute of Physiologically Active Substances of the Russian Academy of Sciences.

The analysis of high-throughput sequencing data was carried out on the equipment of the Genome Shared Use Center of the Engelhardt Institute of Molecular Biology, RAS (http://www.eimb.ru/rus/ckp/ccu_genome_c.php).

Funding

The study of neurodegenerative processes in model animals of the FUS(1-359) line was carried out with the support of the Russian Science Foundation (project no. 19-13-00378).

The animals were kept within the framework of the state assignment of the Institute of Physiologically Active Substances of the Russian Academy of Sciences no. 0090-2017-0019.

Studies of the properties of lipids in neuropathology were carried out within the framework of the state assignment of the Institute of Physiologically Active Substances of the Russian Academy of Sciences (topic no. 44.4, state registration number 01201253310).

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Authors and Affiliations

  1. Emanuel Institute of Biochemical Physics, Russian Academy of Sciences, 119991, Moscow, Russia

    M. A. Shupik, U. A. Gutner & A. V. Alessenko

  2. Institute of Physiologically Active Substances, Russian Academy of Sciences, 142432, Chernogolovka, Russia

    A. A. Ustyugov

  3. Engelhardt Institute of Molecular Biology, 119991, Moscow, Russia

    A. P. Rezvykh & S. Yu. Funikov

  4. Moscow State University, 119999, Moscow, Russia

    O. A. Maloshitskaya, S. A. Sokolov & A. T. Lebedev

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  1. M. A. Shupik
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Translated by N. Onishchenko

Abbreviations: ALS, amyotrophic lateral sclerosis; GalCER, galactosylceramide; SphL, sphingolipids; SM, sphingomyelin; CER, ceramide; 3-KSR, 3-ketodihydrosphyngoreductase; Asah, ceramidase; Asah1, acid ceramidase; aSMase, acid sphingomyelinase; CerS, ceramide synthase; DES, dihydroceramide desaturase; FUS(1-359), FUS transgenic mice; GALC, galactosylceramidase; nSMase, neutral sphingomyelinase; SMase, sphingomyelinase; SMS, sphingomyelinase synthase; SOD1, superoxide dismutase; SPT, serine palmitoyl transferase.

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Shupik, M.A., Gutner, U.A., Ustyugov, A.A. et al. Changes in the Metabolism of Sphingomyelin and Ceramide in the Brain Structures and Spinal Cord of Transgenic Mice (FUS(1-359)) Modeling Amyotrophic Lateral Sclerosis. Russ J Bioorg Chem 48, 178–189 (2022). https://doi.org/10.1134/S1068162022010137

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