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Literature summary extracted from

  • Kreutz, F.; dos Santos Petry, F.; Camassola, M.; Schein, V.; Guma, F.C.; Nardi, N.B.; Trindade, V.M.
    Alterations of membrane lipids and in gene expression of ganglioside metabolism in different brain structures in a mouse model of mucopolysaccharidosis type I (MPS I) (2013), Gene, 527, 109-114.
    View publication on PubMed

Cloned(Commentary)

EC Number Cloned (Comment) Organism
2.4.1.92 gene B4GALNT1, semi-quantitative PCR enzyme expression analysis in brain tissues in wild-type and IDUA knockout mice Mus musculus
2.4.3.8 gene ST8SIA1, semi-quantitative PCR enzyme expression analysis in brain tissues in wild-type and IDUA knockout mice Mus musculus
2.4.3.9 gene St3gal5, semi-quantitative PCR enzyme expression analysis in brain tissues of wild-type and IDUA knockout mice Mus musculus

Protein Variants

EC Number Protein Variants Comment Organism
2.4.1.92 additional information comparisons of relative expressions of ganglioside metabolism genes in wild-type and IDUA knockout mice, overview Mus musculus
2.4.3.8 additional information comparisons of relative expressions of ganglioside metabolism genes in wild-type and IDUA knockout mice, overview Mus musculus
2.4.3.9 additional information comparisons of relative expressions of ganglioside metabolism genes in wild-type and IDUA knockout mice, overview Mus musculus

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
2.4.3.8 CMP-N-acetylneuraminate + ganglioside GM3 Mus musculus
-
 CMP + ganglioside GD3
-
 ?
2.4.3.8 CMP-N-acetylneuraminate + ganglioside GM3 Mus musculus C57BL/6
-
 CMP + ganglioside GD3
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
2.4.1.92 Mus musculus Q09200 alpha-L-iduronidase (IDUA) knockout mutant variant, gene B4GALNT1
-
2.4.1.92 Mus musculus C57BL/6 Q09200 alpha-L-iduronidase (IDUA) knockout mutant variant, gene B4GALNT1
-
2.4.3.8 Mus musculus Q64687 alpha-L-iduronidase (IDUA) knockout mutant variant, gene ST8SIA1
-
2.4.3.8 Mus musculus C57BL/6 Q64687 alpha-L-iduronidase (IDUA) knockout mutant variant, gene ST8SIA1
-
2.4.3.9 Mus musculus O88829 alpha-L-iduronidase (IDUA) knockout mutant variant, gene St3gal5
-
2.4.3.9 Mus musculus C57BL/6 O88829 alpha-L-iduronidase (IDUA) knockout mutant variant, gene St3gal5
-

Source Tissue

EC Number Source Tissue Comment Organism Textmining
2.4.1.92 hippocampus
-
 Mus musculus
-
2.4.1.92 hypothalamus
-
 Mus musculus
-
2.4.1.92 additional information ganglioside profile analyses in brain tissues, overview. Tissue dependent ganglioside alteration in IDUA KO mice with a total ganglioside increase in cortex and cerebellum, and a selective presence of GM3, GM2 and GD3 gangliosides in the hippocampus and hypothalamus. Evaluation of gene expression of ganglioside synthesis (GM3, GD3 and GM2/GD2 synthases) and degradation of (neuraminidase1) enzymes in the cerebellum and hippocampus by RT-sq-PCR. Percentage distribution of gangliosides in wild-type and IDUA knockout mice, overview Mus musculus
-
2.4.3.8 hippocampus
-
 Mus musculus
-
2.4.3.8 hypothalamus
-
 Mus musculus
-
2.4.3.8 additional information ganglioside profile analyses in brain tissues, overview. Tissue dependent ganglioside alteration in IDUA KO mice with a total ganglioside increase in cortex and cerebellum, and a selective presence of GM3, GM2 and GD3 gangliosides in the hippocampus and hypothalamus. Evaluation of gene expression of ganglioside synthesis (GM3, GD3 and GM2/GD2 synthases) and degradation of (neuraminidase1) enzymes in the cerebellum and hippocampus by RT-sq-PCR. Percentage distribution of gangliosides in wild-type and IDUA knockout mice, overview Mus musculus
-
2.4.3.9 hippocampus
-
 Mus musculus
-
2.4.3.9 hypothalamus
-
 Mus musculus
-
2.4.3.9 additional information ganglioside profile analyses in brain tissues, overview. Tissue dependent ganglioside alteration in IDUA KO mice with a total ganglioside increase in cortex and cerebellum, and a selective presence of GM3, GM2 and GD3 gangliosides in the hippocampus and hypothalamus. Evaluation of gene expression of ganglioside synthesis (GM3, GD3 and GM2/GD2 synthases) and degradation of (neuraminidase1) enzymes in the cerebellum and hippocampus by RT-sq-PCR. Percentage distribution of gangliosides in wild-type and IDUA knockout mice, overview Mus musculus
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
2.4.3.8 CMP-N-acetylneuraminate + ganglioside GM3
-
 Mus musculus CMP + ganglioside GD3
-
 ?
2.4.3.8 CMP-N-acetylneuraminate + ganglioside GM3
-
 Mus musculus C57BL/6 CMP + ganglioside GD3
-
 ?

Synonyms

EC Number Synonyms Comment Organism
2.4.1.92 GM2/GD2 synthase
-
 Mus musculus
2.4.3.8 GD3 synthase
-
 Mus musculus
2.4.3.9 GM3 synthase
-
 Mus musculus

General Information

EC Number General Information Comment Organism
2.4.1.92 malfunction alpha-L-iduronidase knockout mutant, IDUA KO, mice show reduced expression of GD3 and GM2/GD2 synthases and neuraminidase1 in cerebellum, and a decrease in GM2/GD2 synthase and neuraminidase 1 in the hippocampus. The observed ganglioside changes result from a combined effect of glycosaminoglycans on ganglioside biosynthesis and degradation. C57Bl/6 knockout mice deficient for alpha-L-iduronidase (IDUA-KO) represent a murine model for human mucopolysaccharidosis type I, MPS I, an autosomal recessive disease caused by a genetic defect that codifies a lysosomal hydrolase, alpha-L-iduronidase, IDUA, EC. 3.2.1.76 Mus musculus
2.4.1.92 metabolism the enzyme is responsible for the biosynthesis of GM2 and GD2 gangliosides, biosynthesis and degradation pathways of a- and b-series gangliosides, overview Mus musculus
2.4.3.8 malfunction alpha-L-iduronidase knockout mutant, IDUA KO, mice show reduced expression of GD3 and GM2/GD2 synthases and neuraminidase1 in cerebellum, and a decrease in GM2/GD2 synthase and neuraminidase1 in the hippocampus. The observed ganglioside changes result from a combined effect of glycosaminoglycans on ganglioside biosynthesis and degradation. C57Bl/6 knockout mice deficient for alpha-L-iduronidase (IDUA-KO) represent a murine model for human mucopolysaccharidosis type I, MPS I, an autosomal recessive disease caused by a genetic defect that codifies a lysosomal hydrolase, alpha-L-iduronidase, IDUA, EC. 3.2.1.76 Mus musculus
2.4.3.8 metabolism the enzyme is responsible for the biosynthesis of GD3 ganglioside, biosynthesis and degradation pathways of a- and b-series gangliosides, overview Mus musculus
2.4.3.9 malfunction alpha-L-iduronidase knockout mutant , IDUA KO, mice show reduced expression of GD3 and GM2/GD2 synthases and neuraminidase1 in cerebellum, and a decrease in GM2/GD2 synthase and neuraminidase 1 in the hippocampus. The observed ganglioside changes result from a combined effect of glycosaminoglycans on ganglioside biosynthesis and degradation. C57Bl/6 knockout mice deficient for alpha-L-iduronidase (IDUA-KO) represent a murine model for human mucopolysaccharidosis type I, MPS I, an autosomal recessive disease caused by a genetic defect that codifies a lysosomal hydrolase, alpha-L-iduronidase, IDUA, EC. 3.2.1.76 Mus musculus
2.4.3.9 metabolism the enzyme is responsible for the biosynthesis of GM3 ganglioside, biosynthesis and degradation pathways of a- and b-series gangliosides, overview Mus musculus