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Literature summary for 3.1.6.12 extracted from

  • O'Brien, J.F.; Cantz, M.; Spranger, J.
    Maroteaux-Lamy disease (mucopolysaccharidosis VI), subtype A: deficiency of a N-acetylgalactosamine-4-sulfatase (1974), Biochem. Biophys. Res. Commun., 60, 1170-1177.
    View publication on PubMed

Application

Application Comment Organism
medicine deficiency in Maroteaux-Lamy syndrome, mucopolysaccharidosis type IV Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
N-acetyl-D-galactosamine 4-sulfate units + H2O Homo sapiens acts also on N-acetylglucosamine 4-sulfate N-acetyl-D-galactosamine units + sulfate
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?

Organism

Organism UniProt Comment Textmining
Homo sapiens
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-
-

Source Tissue

Source Tissue Comment Organism Textmining
skin cultured fibroblasts Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
dermatan sulfate + H2O reduced activity in patients with Maroteaux-Lamy Syndrome Homo sapiens dermatan + sulfate
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?
N-acetyl-D-galactosamine 4-sulfate units + H2O acts also on N-acetylglucosamine 4-sulfate Homo sapiens N-acetyl-D-galactosamine units + sulfate
-
?