3.10.1.1: N-sulfoglucosamine sulfohydrolase
This is an abbreviated version!
For detailed information about N-sulfoglucosamine sulfohydrolase, go to the full flat file.
Word Map on EC 3.10.1.1
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3.10.1.1
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mucopolysaccharidosis
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lysosomal
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sanfilippo
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mps-iiia
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naglu
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n-sulfate
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sulfate-derived
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heparinum
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medicine
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oestrone
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hgsnat
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sumf1
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iduronate
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sulfatases
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ubiquitin-positive
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drug development
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pharmacology
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diagnostics
- 3.10.1.1
- mucopolysaccharidosis
- lysosomal
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sanfilippo
-
mps-iiia
- naglu
-
n-sulfate
-
sulfate-derived
- heparinum
- medicine
- oestrone
- hgsnat
- sumf1
-
iduronate
-
sulfatases
-
ubiquitin-positive
- drug development
- pharmacology
- diagnostics
Reaction
Synonyms
2-desoxy-2-sulphamido-D-glucose sulphamidase, 2-desoxy-D-glucoside-2-sulphamate sulphohydrolase, heparan N-sulfatase, heparan-N-sulfatase, heparan-N-sulphatase, heparin sulfamidase, N-sulfoglucosamine sulfohydrolase, N-sulphoglucosamine, SGSH, sulfamate sulfohydrolase, sulfamidase, sulfoglucosamine sulfamidase, sulphamate sulphohydrolase, sulphamidase, sulphohydrolase
ECTree
3.10.1.1 N-sulfoglucosamine sulfohydrolaseAdvanced search results
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results (Substrates Products
Substrates Products on EC 3.10.1.1 - N-sulfoglucosamine sulfohydrolase
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SUBSTRATE 
PRODUCT
REACTION DIAGRAM
ORGANISM
UNIPROT
COMMENTARY
(Substrate)
(Substrate)
LITERATURE
(Substrate)
(Substrate)
COMMENTARY
(Product)
(Product)
LITERATURE
(Product)
(Product)
Reversibility
r=reversible
ir=irreversible
?=not specified
r=reversible
ir=irreversible
?=not specified
2-deoxy-2-sulfamido-D-glucose + H2O
2-deoxy-2-amino-D-glucose + sulfate
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ir
4-methylumbelliferyl-alpha-D-N-sulfoglucosaminide + H2O
4-methylumbelliferyl alpha-D-glucosaminide + sulfate
O-(alpha-2-sulfamino-2-deoxy-D-glucopyranosyl)-(1-3)-L-idonic acid
O-(alpha-2-amino-2-deoxy-D-glucopyranosyl)-(1-3)-L-idonic acid + sulfate
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?
4-methylumbelliferyl-alpha-D-N-sulfoglucosaminide + H2O
4-methylumbelliferyl alpha-D-glucosaminide + sulfate
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?
4-methylumbelliferyl-alpha-D-N-sulfoglucosaminide + H2O
4-methylumbelliferyl alpha-D-glucosaminide + sulfate
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?
4-methylumbelliferyl-alpha-D-N-sulfoglucosaminide + H2O
4-methylumbelliferyl alpha-D-glucosaminide + sulfate
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?
glucosamine 2,6-disulfate + H2O
D-glucosamine 6-sulfate + sulfate
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ir
glucosamine 2,6-disulfate + H2O
D-glucosamine 6-sulfate + sulfate
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no activity with purified enzyme
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additional information
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enzyme defiency leads to defective lysosomal degradation of the glycosaminoglycan heparan sulfate, mutations of the enzyme are responsible for mucopolysaccharidosis type IIIA, i.e. Sanfilippo A syndrome, onset and progression of the disease, overview
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additional information
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reduced activity of sulfamidase results in intracellular accumulation of heparan sulfate, with the brain the primary site of pathology, e.g. in mucopolysaccharidosis type IIIA, or Sanfilippo syndrome, an inherited neurodegenerative lysosomal storage disorder, progressive loss of learned skills, sleep disturbance and behavioural problems occur. A potential therapy method is the repeated injection of the enzyme into cerebrospinal fluid via cisterna magna leading to a reduction of the number of lysosomal storage inclusions in the brain with a significant decrease in the immunohistochemical staining of a lysosomal membrane marker, and to reduced numbers of activated isolectin-B4-positive microglia and GFAP-positive astrocytes in some brain regions, phenotype, mouse model, overview
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additional information
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repeated human enzyme injection into murine cerebrospinal fluid via cisterna magna leads to a reduction of heparan sulfate-derived monosulfated disaccharide in the brain and spinal cord, reduced lysosomal vesicle formation in various cell types, reduced axonal spheroids, and improved behaviour of treated mice, mouse model, overview
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additional information
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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, which converts 4-methylumbelliferyl-alpha-D-N-sulfoglucosamine to methylumbelliferone, which is a fluorescent compound
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additional information
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congenital deficiency of sulfamidase leads to mucopolysaccharidosis type IIIA or Sanfilippo syndrome, a lysosomal storage disorder, with consequent accumulation of partially degraded heparan sulfate in lysosomes and the central nervous system as the predominant site of tissue damage, overview
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additional information
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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
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additional information
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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
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