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Literature summary for 3.1.6.12 extracted from

  • Matalon, R.; Arbogast, B.; Dorfmann, A.
    Deficiency of chondroitin sulfate N-acetylgalactosamine 4-sulfate sulfatase in Maroteaux-Lamy syndrome (1974), Biochem. Biophys. Res. Commun., 61, 1450-1457.
    View publication on PubMed
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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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-
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Source Tissue

Source Tissue Comment Organism Textmining
skin cultured fibroblasts Homo sapiens
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Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
chondroitin 4-sulfate + H2O
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 Homo sapiens chondroitin + sulfate
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 ?
chondroitin 4-sulfate-heptasaccharide + H2O reduced activity in patients with Maroteaux-Lamy Syndrome Homo sapiens chondroitin 4-sulfate-heptasaccharide + 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
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 ?