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Literature summary for 2.3.1.78 extracted from

  • Voznyi, Y.V.; Karpova, E.A.; Dudukina, T.V.; Tsvetkova, I.V.; Boer, A.M.; Janse, H.C.; van Diggelen, O.P.
    A fluorometric enzyme assay for the diagnosis of Sanfilippo disease C (MPS III C) (1993), J. Inherit. Metab. Dis., 16, 465-472.
    View publication on PubMed

Activating Compound

Activating Compound Comment Organism Structure
Triton X-100 1.5 to 2.5fold stimulation at 0.25% w/v Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
lysosome
-
Homo sapiens 5764
-

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
acetyl-CoA + heparan sulfate alpha-D-glucosaminide Homo sapiens
-
CoA + heparan sulfate N-acetyl-alpha-D-glucosaminide
-
?
additional information Homo sapiens enzyme deficiency leads to Sanfilippo syndrome type C, i.e. mucopolysaccharidosis III C ?
-
?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
leukocyte deficiency on leukocytes from patients with Sanfillippo syndrome type C Homo sapiens
-
skin fibroblast deficiency on fibroblasts from patients with Sanfillippo syndrome type C Homo sapiens
-

Specific Activity [micromol/min/mg]

Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
additional information
-
Sanfilippo syndrome type C patients in comparison Homo sapiens
0.000017
-
leukocyte, substrate 4-methylumbelliferyl-alpha-D-glucosaminide + acetyl-CoA Homo sapiens
0.000022
-
leukocyte, substrate 4-methylumbelliferyl-beta-D-glucosaminide + acetyl-CoA Homo sapiens
0.000058
-
fibroblast, substrate 4-methylumbelliferyl-beta-D-glucosaminide + acetyl-CoA Homo sapiens
0.000059
-
fibroblast, substrate 4-methylumbelliferyl-alpha-D-glucosaminide + acetyl-CoA Homo sapiens

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
acetyl-CoA + 4-methylumbelliferyl-alpha-D-glucosaminide
-
Homo sapiens CoA + 4-methylumbelliferyl-N-acetyl-alpha-D-glucosaminide
-
?
acetyl-CoA + 4-methylumbelliferyl-beta-D-glucosaminide
-
Homo sapiens CoA + 4-methylumbelliferyl-N-acetyl-beta-D-glucosaminide
-
?
acetyl-CoA + heparan sulfate alpha-D-glucosaminide
-
Homo sapiens CoA + heparan sulfate N-acetyl-alpha-D-glucosaminide
-
?
additional information enzyme deficiency leads to Sanfilippo syndrome type C, i.e. mucopolysaccharidosis III C Homo sapiens ?
-
?

Temperature Optimum [°C]

Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
37
-
assay at Homo sapiens

pH Optimum

pH Optimum Minimum pH Optimum Maximum Comment Organism
5.7
-
-
Homo sapiens