Dietary gangliosides rescue GM3 synthase deficiency outcomes in mice accompanied by neurogenesis in the hippocampus
Ganglioside GM3 synthase is a key enzyme involved in the biosynthesis of gangliosides. GM3 synthase deficiency (GM3SD) causes an absence of GM3 and all downstream biosynthetic derivatives, including all the a-, b-, c-series gangliosides, commonly found in neural tissues. The affected individuals man...
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| Published in | Frontiers in neuroscience Vol. 18; p. 1387221 |
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| Main Authors | , , , , , , , , , , , |
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| Language | English |
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25.07.2024
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| Abstract | Ganglioside GM3 synthase is a key enzyme involved in the biosynthesis of gangliosides. GM3 synthase deficiency (GM3SD) causes an absence of GM3 and all downstream biosynthetic derivatives, including all the a-, b-, c-series gangliosides, commonly found in neural tissues. The affected individuals manifest with severe irritability, intractable seizures, hearing loss, blindness, and profound intellectual disability. It has been reported that oral ganglioside supplementation has achieved some significant improvements in clinical symptoms, growth parameters, and developmental and cognitive scores in GM3SD patients. To gain insight into the molecular mechanisms of this supplementation, we performed supplementation of oral bovine milk gangliosides to GM3 synthase-deficient mice from early weaning periods. The oral milk ganglioside preparations were dominated by GM3 and GD3 gangliosides. Oral milk ganglioside supplementation improved the decreased cognitive function observed in GM3 synthase-deficient mice. The improvement in cognitive function was accompanied by increased ganglioside levels and neurogenesis in the hippocampus in the supplemented animals. |
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| AbstractList | Ganglioside GM3 synthase is a key enzyme involved in the biosynthesis of gangliosides. GM3 synthase deficiency (GM3SD) causes an absence of GM3 and all downstream biosynthetic derivatives, including all the a-, b-, c-series gangliosides, commonly found in neural tissues. The affected individuals manifest with severe irritability, intractable seizures, hearing loss, blindness, and profound intellectual disability. It has been reported that oral ganglioside supplementation has achieved some significant improvements in clinical symptoms, growth parameters, and developmental and cognitive scores in GM3SD patients. To gain insight into the molecular mechanisms of this supplementation, we performed supplementation of oral bovine milk gangliosides to GM3 synthase-deficient mice from early weaning periods. The oral milk ganglioside preparations were dominated by GM3 and GD3 gangliosides. Oral milk ganglioside supplementation improved the decreased cognitive function observed in GM3 synthase-deficient mice. The improvement in cognitive function was accompanied by increased ganglioside levels and neurogenesis in the hippocampus in the supplemented animals. Ganglioside GM3 synthase is a key enzyme involved in the biosynthesis of gangliosides. GM3 synthase deficiency (GM3SD) causes an absence of GM3 and all downstream biosynthetic derivatives, including all the a-, b-, c-series gangliosides, commonly found in neural tissues. The affected individuals manifest with severe irritability, intractable seizures, hearing loss, blindness, and profound intellectual disability. It has been reported that oral ganglioside supplementation has achieved some significant improvements in clinical symptoms, growth parameters, and developmental and cognitive scores in GM3SD patients. To gain insight into the molecular mechanisms of this supplementation, we performed supplementation of oral bovine milk gangliosides to GM3 synthase-deficient mice from early weaning periods. The oral milk ganglioside preparations were dominated by GM3 and GD3 gangliosides. Oral milk ganglioside supplementation improved the decreased cognitive function observed in GM3 synthase-deficient mice. The improvement in cognitive function was accompanied by increased ganglioside levels and neurogenesis in the hippocampus in the supplemented animals.Ganglioside GM3 synthase is a key enzyme involved in the biosynthesis of gangliosides. GM3 synthase deficiency (GM3SD) causes an absence of GM3 and all downstream biosynthetic derivatives, including all the a-, b-, c-series gangliosides, commonly found in neural tissues. The affected individuals manifest with severe irritability, intractable seizures, hearing loss, blindness, and profound intellectual disability. It has been reported that oral ganglioside supplementation has achieved some significant improvements in clinical symptoms, growth parameters, and developmental and cognitive scores in GM3SD patients. To gain insight into the molecular mechanisms of this supplementation, we performed supplementation of oral bovine milk gangliosides to GM3 synthase-deficient mice from early weaning periods. The oral milk ganglioside preparations were dominated by GM3 and GD3 gangliosides. Oral milk ganglioside supplementation improved the decreased cognitive function observed in GM3 synthase-deficient mice. The improvement in cognitive function was accompanied by increased ganglioside levels and neurogenesis in the hippocampus in the supplemented animals. |
| Author | Inamori, Kei-Ichiro Tan-No, Koichi Collett, Michael Go, Shinji McJarrow, Paul Nakagawasai, Osamu Veillon, Lucas Odaira-Satoh, Takayo Nitta, Takahiro Suzuki, Akemi Inokuchi, Jin-Ichi Kanoh, Hirotaka |
| AuthorAffiliation | 1 Division of Glycopathology, Institute of Molecular Biomembranes and Glycobiology, Tohoku Medical and Pharmaceutical University , Sendai , Japan 2 Forefront Research Centre, Graduate School of Science, Osaka University , Toyonaka , Japan 3 Division of Pharmacology, Faculty of Pharmaceutical Sciences, Tohoku Medical and Pharmaceutical University , Sendai , Japan 4 Fonterra Research and Development Centre , Palmerston North , New Zealand |
| AuthorAffiliation_xml | – name: 1 Division of Glycopathology, Institute of Molecular Biomembranes and Glycobiology, Tohoku Medical and Pharmaceutical University , Sendai , Japan – name: 2 Forefront Research Centre, Graduate School of Science, Osaka University , Toyonaka , Japan – name: 3 Division of Pharmacology, Faculty of Pharmaceutical Sciences, Tohoku Medical and Pharmaceutical University , Sendai , Japan – name: 4 Fonterra Research and Development Centre , Palmerston North , New Zealand |
| Author_xml | – sequence: 1 givenname: Jin-Ichi surname: Inokuchi fullname: Inokuchi, Jin-Ichi organization: Forefront Research Centre, Graduate School of Science, Osaka University, Toyonaka, Japan – sequence: 2 givenname: Shinji surname: Go fullname: Go, Shinji organization: Division of Glycopathology, Institute of Molecular Biomembranes and Glycobiology, Tohoku Medical and Pharmaceutical University, Sendai, Japan – sequence: 3 givenname: Akemi surname: Suzuki fullname: Suzuki, Akemi organization: Division of Glycopathology, Institute of Molecular Biomembranes and Glycobiology, Tohoku Medical and Pharmaceutical University, Sendai, Japan – sequence: 4 givenname: Osamu surname: Nakagawasai fullname: Nakagawasai, Osamu organization: Division of Pharmacology, Faculty of Pharmaceutical Sciences, Tohoku Medical and Pharmaceutical University, Sendai, Japan – sequence: 5 givenname: Takayo surname: Odaira-Satoh fullname: Odaira-Satoh, Takayo organization: Division of Pharmacology, Faculty of Pharmaceutical Sciences, Tohoku Medical and Pharmaceutical University, Sendai, Japan – sequence: 6 givenname: Lucas surname: Veillon fullname: Veillon, Lucas organization: Division of Glycopathology, Institute of Molecular Biomembranes and Glycobiology, Tohoku Medical and Pharmaceutical University, Sendai, Japan – sequence: 7 givenname: Takahiro surname: Nitta fullname: Nitta, Takahiro organization: Division of Glycopathology, Institute of Molecular Biomembranes and Glycobiology, Tohoku Medical and Pharmaceutical University, Sendai, Japan – sequence: 8 givenname: Paul surname: McJarrow fullname: McJarrow, Paul organization: Fonterra Research and Development Centre, Palmerston North, New Zealand – sequence: 9 givenname: Hirotaka surname: Kanoh fullname: Kanoh, Hirotaka organization: Division of Glycopathology, Institute of Molecular Biomembranes and Glycobiology, Tohoku Medical and Pharmaceutical University, Sendai, Japan – sequence: 10 givenname: Kei-Ichiro surname: Inamori fullname: Inamori, Kei-Ichiro organization: Division of Glycopathology, Institute of Molecular Biomembranes and Glycobiology, Tohoku Medical and Pharmaceutical University, Sendai, Japan – sequence: 11 givenname: Koichi surname: Tan-No fullname: Tan-No, Koichi organization: Division of Pharmacology, Faculty of Pharmaceutical Sciences, Tohoku Medical and Pharmaceutical University, Sendai, Japan – sequence: 12 givenname: Michael surname: Collett fullname: Collett, Michael organization: Fonterra Research and Development Centre, Palmerston North, New Zealand |
| BackLink | https://www.ncbi.nlm.nih.gov/pubmed/39119456$$D View this record in MEDLINE/PubMed |
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| Copyright | Copyright © 2024 Inokuchi, Go, Suzuki, Nakagawasai, Odaira-Satoh, Veillon, Nitta, McJarrow, Kanoh, Inamori, Tan-No and Collett. Copyright © 2024 Inokuchi, Go, Suzuki, Nakagawasai, Odaira-Satoh, Veillon, Nitta, McJarrow, Kanoh, Inamori, Tan-No and Collett. 2024 Inokuchi, Go, Suzuki, Nakagawasai, Odaira-Satoh, Veillon, Nitta, McJarrow, Kanoh, Inamori, Tan-No and Collett |
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| Keywords | cognitive function milk gangliosides hippocampus oral supplementation GD3 neurogenesis GM3 synthase deficiency GM3 |
| Language | English |
| License | Copyright © 2024 Inokuchi, Go, Suzuki, Nakagawasai, Odaira-Satoh, Veillon, Nitta, McJarrow, Kanoh, Inamori, Tan-No and Collett. This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms. |
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| SubjectTerms | GD3 GM3 GM3 synthase deficiency milk gangliosides neurogenesis Neuroscience oral supplementation |
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| Title | Dietary gangliosides rescue GM3 synthase deficiency outcomes in mice accompanied by neurogenesis in the hippocampus |
| URI | https://www.ncbi.nlm.nih.gov/pubmed/39119456 https://www.proquest.com/docview/3090946735 https://pubmed.ncbi.nlm.nih.gov/PMC11308210 https://doaj.org/article/caa20b818c9a4c83b5ef00dcc0053041 |
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