g(m3)-ganglioside and Seizures

g(m3)-ganglioside has been researched along with Seizures* in 2 studies

Other Studies

2 other study(ies) available for g(m3)-ganglioside and Seizures

Article	Year
GM3 synthase deficiency increases brain glucose metabolism in mice. Molecular genetics and metabolism, 2022, Volume: 137, Issue:4 GM3 synthase (GM3S) deficiency is a rare neurodevelopmental disorder caused by an inability to synthesize gangliosides, for which there is currently no treatment. Gangliosides are brain-enriched, plasma membrane glycosphingolipids with poorly understood biological functions related to cell adhesion, growth, and receptor-mediated signal transduction. Here, we investigated the effects of GM3S deficiency on metabolism and mitochondrial function in a mouse model. By indirect calorimetry, GM3S knockout mice exhibited increased whole-body respiration and an increased reliance upon carbohydrate as an energy source. Topics: Animals; Brain; G(M3) Ganglioside; Glucose; Mice; Mice, Knockout; Pyruvic Acid; Seizures; Sialyltransferases	2022
Mice expressing only monosialoganglioside GM3 exhibit lethal audiogenic seizures. The Journal of biological chemistry, 2001, Mar-09, Volume: 276, Issue:10 Gangliosides are a family of glycosphingolipids that contain sialic acid. Although they are abundant on neuronal cell membranes, their precise functions and importance in the central nervous system (CNS) remain largely undefined. We have disrupted the gene encoding GD3 synthase (GD3S), a sialyltransferase expressed in the CNS that is responsible for the synthesis of b-series gangliosides. GD3S-/- mice, even with an absence of b-series gangliosides, appear to undergo normal development and have a normal life span. To further restrict the expression of gangliosides, the GD3S mutant mice were crossbred with mice carrying a disrupted GalNAcT gene encoding beta1,4-N-acetylgalactosaminyltransferase. These double mutant mice expressed GM3 as their major ganglioside. In contrast to the single mutant mice, the double mutants displayed a sudden death phenotype and were extremely susceptible to induction of lethal seizures by sound stimulus. These results demonstrate unequivocally that gangliosides play an essential role in the proper functioning of the CNS. Topics: Age Factors; Animals; Central Nervous System; Crosses, Genetic; G(M3) Ganglioside; Gangliosides; Gene Library; Glycosyltransferases; Mice; Mice, Knockout; Models, Biological; Models, Genetic; Mutation; N-Acetylgalactosaminyltransferases; Phenotype; Seizures; Sialyltransferases	2001

Article

Year

GM3 synthase deficiency increases brain glucose metabolism in mice.

Molecular genetics and metabolism, 2022, Volume: 137, Issue:4

GM3 synthase (GM3S) deficiency is a rare neurodevelopmental disorder caused by an inability to synthesize gangliosides, for which there is currently no treatment. Gangliosides are brain-enriched, plasma membrane glycosphingolipids with poorly understood biological functions related to cell adhesion, growth, and receptor-mediated signal transduction. Here, we investigated the effects of GM3S deficiency on metabolism and mitochondrial function in a mouse model. By indirect calorimetry, GM3S knockout mice exhibited increased whole-body respiration and an increased reliance upon carbohydrate as an energy source.

Topics: Animals; Brain; G(M3) Ganglioside; Glucose; Mice; Mice, Knockout; Pyruvic Acid; Seizures; Sialyltransferases

2022

Mice expressing only monosialoganglioside GM3 exhibit lethal audiogenic seizures.

The Journal of biological chemistry, 2001, Mar-09, Volume: 276, Issue:10

Gangliosides are a family of glycosphingolipids that contain sialic acid. Although they are abundant on neuronal cell membranes, their precise functions and importance in the central nervous system (CNS) remain largely undefined. We have disrupted the gene encoding GD3 synthase (GD3S), a sialyltransferase expressed in the CNS that is responsible for the synthesis of b-series gangliosides. GD3S-/- mice, even with an absence of b-series gangliosides, appear to undergo normal development and have a normal life span. To further restrict the expression of gangliosides, the GD3S mutant mice were crossbred with mice carrying a disrupted GalNAcT gene encoding beta1,4-N-acetylgalactosaminyltransferase. These double mutant mice expressed GM3 as their major ganglioside. In contrast to the single mutant mice, the double mutants displayed a sudden death phenotype and were extremely susceptible to induction of lethal seizures by sound stimulus. These results demonstrate unequivocally that gangliosides play an essential role in the proper functioning of the CNS.

Topics: Age Factors; Animals; Central Nervous System; Crosses, Genetic; G(M3) Ganglioside; Gangliosides; Gene Library; Glycosyltransferases; Mice; Mice, Knockout; Models, Biological; Models, Genetic; Mutation; N-Acetylgalactosaminyltransferases; Phenotype; Seizures; Sialyltransferases

2001