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Literature summary for 2.3.1.78 extracted from

  • Klein, U.; Kresse, H.; Von Figura, K.
    Sanfilippo syndrome type C: deficiency of acetyl-CoA: alpha-glucosaminide N-acetyltransferase in skin fibroblasts (1978), Proc. Natl. Acad. Sci. USA, 75, 5185-5189.
    View publication on PubMedView publication on EuropePMC

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 initial step in heparan sulfate degradation 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
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
-
-
Homo sapiens

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
acetyl-CoA + heparan sulfate alpha-D-glucosaminide brings about the acetylation of glucosamine groups of heparan sulfate and heparin from which the sulfate has been removed Homo sapiens CoA + heparan sulfate N-acetyl-alpha-D-glucosaminide
-
?
acetyl-CoA + heparan sulfate alpha-D-glucosaminide initial step in heparan sulfate degradation 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
5.5
-
about Homo sapiens

pH Range

pH Minimum pH Maximum Comment Organism
5 7 half-maximal activity at pH 5.0 and pH 7.0 Homo sapiens