Application | Comment | Organism |
---|---|---|
diagnostics | a fluorimetric SGSH activity assay is commonly used to examine cells of mucopolysaccharidosis type IIIA patients for N-sulfoglucosamine sulfohydrolase activity, modification of the method for brain homogenates and definition of the parameters for assay linearity, overview | Homo sapiens |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
Mus musculus | - |
- |
- |
Mus musculus C57BL/6 | - |
- |
- |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
brain | - |
Mus musculus | - |
brain | - |
Homo sapiens | - |
Substrates | Comment Substrates | Organism | Products | Comment (Products) | Rev. | Reac. |
---|---|---|---|---|---|---|
4-methylumbelliferyl-alpha-D-N-sulfoglucosaminide + H2O | - |
Mus musculus | 4-methylumbelliferyl alpha-D-glucosaminide + sulfate | - |
? | |
4-methylumbelliferyl-alpha-D-N-sulfoglucosaminide + H2O | - |
Mus musculus C57BL/6 | 4-methylumbelliferyl alpha-D-glucosaminide + sulfate | - |
? | |
additional information | the enzymatic activity of the enzyme is measured in a two-step reaction: 4-methylumbelliferyl-alpha-D-N-sulfoglucosaminide is desulfated by the enzyme to become a substrate for alpha-glucosidase from Bacillus stearothermophilus, which converts 4-methylumbelliferyl-alpha-D-N-sulfoglucosamine to methylumbelliferone, which is a fluorescent compound. Method evaluation | Mus musculus | ? | - |
? | |
additional information | the enzymatic activity of the enzyme is measured in a two-step reaction: 4-methylumbelliferyl-alpha-D-N-sulfoglucosaminide is desulfated by the enzyme to become a substrate for alpha-glucosidase from Bacillus stearothermophilus, which converts 4-methylumbelliferyl-alpha-D-N-sulfoglucosamine to methylumbelliferone, which is a fluorescent compound. Method evaluation | Mus musculus C57BL/6 | ? | - |
? |
Synonyms | Comment | Organism |
---|---|---|
SGSH | - |
Mus musculus |
SGSH | - |
Homo sapiens |
Temperature Optimum [°C] | Temperature Optimum Maximum [°C] | Comment | Organism |
---|---|---|---|
37 | - |
assay at | Mus musculus |
pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
---|---|---|---|
6.7 | - |
assay at | Mus musculus |
General Information | Comment | Organism |
---|---|---|
malfunction | deficient N-sulfoglucosamine sulfohydrolase enzyme activity causes mucopolysaccharidosis (MPS) type IIIA | Mus musculus |
malfunction | deficient N-sulfoglucosamine sulfohydrolase enzyme activity causes mucopolysaccharidosis (MPS) type IIIA | Homo sapiens |