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Literature summary for 3.10.1.1 extracted from

  • Whyte, L.; Hopwood, J.; Hemsley, K.; Lau, A.
    Variables influencing fluorimetric N-sulfoglucosamine sulfohydrolase (SGSH) activity measurement in brain homogenates (2015), Mol. Genet. Metab. Rep., 5, 60-62.
    View publication on PubMedView publication on EuropePMC
Search for more literature for 3.10.1.1

Application

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

Organism UniProt Comment Textmining
Homo sapiens
-
-
-
Mus musculus
-
-
-
Mus musculus C57BL/6
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
brain
-
 Mus musculus
-
brain
-
 Homo sapiens
-

Substrates and Products (Substrate)

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

Synonyms Comment Organism
SGSH
-
 Mus musculus
SGSH
-
 Homo sapiens

Temperature Optimum [°C]

Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
37
-
 assay at Mus musculus

pH Optimum

pH Optimum Minimum pH Optimum Maximum Comment Organism
6.7
-
 assay at Mus musculus

General Information

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