Cloned (Comment) | Organism |
---|---|
gene ST8SIA1, semi-quantitative PCR enzyme expression analysis in brain tissues in wild-type and IDUA knockout mice | Mus musculus |
Protein Variants | Comment | Organism |
---|---|---|
additional information | comparisons of relative expressions of ganglioside metabolism genes in wild-type and IDUA knockout mice, overview | Mus musculus |
Natural Substrates | Organism | Comment (Nat. Sub.) | Natural Products | Comment (Nat. Pro.) | Rev. | Reac. |
---|---|---|---|---|---|---|
CMP-N-acetylneuraminate + ganglioside GM3 | Mus musculus | - |
CMP + ganglioside GD3 | - |
? | |
CMP-N-acetylneuraminate + ganglioside GM3 | Mus musculus C57BL/6 | - |
CMP + ganglioside GD3 | - |
? |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Mus musculus | Q64687 | alpha-L-iduronidase (IDUA) knockout mutant variant, gene ST8SIA1 | - |
Mus musculus C57BL/6 | Q64687 | alpha-L-iduronidase (IDUA) knockout mutant variant, gene ST8SIA1 | - |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
hippocampus | - |
Mus musculus | - |
hypothalamus | - |
Mus musculus | - |
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 | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
CMP-N-acetylneuraminate + ganglioside GM3 | - |
Mus musculus | CMP + ganglioside GD3 | - |
? | |
CMP-N-acetylneuraminate + ganglioside GM3 | - |
Mus musculus C57BL/6 | CMP + ganglioside GD3 | - |
? |
Synonyms | Comment | Organism |
---|---|---|
GD3 synthase | - |
Mus musculus |
General Information | Comment | Organism |
---|---|---|
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 |
metabolism | the enzyme is responsible for the biosynthesis of GD3 ganglioside, biosynthesis and degradation pathways of a- and b-series gangliosides, overview | Mus musculus |