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Literature summary extracted from

  • Mandelli, J.; Wajner, A.; Pires, R.; Giugliani, R.; Coelho, J.C.
    Effect of CuCl2, NaCl and EDTA on the enzyme alpha-L-iduronidase in the plasma of normal individuals and heterozygotes for MPS I (2002), Clin. Chim. Acta, 318, 83-89.
    View publication on PubMed

Inhibitors

EC Number Inhibitors Comment Organism Structure
3.2.1.76 CuCl2 strong noncompetitive inhibitor for enzyme from healthy individuals and mucopolysaccharidosis type I patients Homo sapiens
3.2.1.76 EDTA competitive, equal inhibition of enzyme from healthy individuals and mucopolysaccharidosis type I patients Homo sapiens
3.2.1.76 NaCl healthy individuals and 40% of heterocygotic mucopolysaccharidosis type I patients, activating for 60% of heterocygotic mucopolysaccharidosis type I patients Homo sapiens

KM Value [mM]

EC Number KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
3.2.1.76 0.21
-
 4-methylumbelliferyl-alpha-L-iduronide pH 2.8, 37°C, enzyme from healthy individuals in the presence of 10 mM CuCl2l Homo sapiens
3.2.1.76 0.22
-
 4-methylumbelliferyl-alpha-L-iduronide pH 2.8, 37°C, enzyme from healthy individuals Homo sapiens
3.2.1.76 0.48
-
 4-methylumbelliferyl-alpha-L-iduronide pH 2.8, 37°C, enzyme from healthy individuals in the presence of 20 mM EDTA Homo sapiens
3.2.1.76 0.81
-
 4-methylumbelliferyl-alpha-L-iduronide pH 2.8, 37°C, enzyme from healthy individuals in the presence of 0.1 M NaCl Homo sapiens

Localization

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

Natural Substrates/ Products (Substrates)

EC Number Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
3.2.1.76 additional information Homo sapiens involved in the degradation of dermatan sulfate and heparan sulfate, deficiency in alpha-L-iduronidase causes mucopolysaccharidosis type I ?
-
 ?

Organism

EC Number Organism UniProt Comment Textmining
3.2.1.76 Homo sapiens
-
-
-

Substrates and Products (Substrate)

EC Number Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
3.2.1.76 4-methylumbelliferyl-alpha-L-iduronide + H2O
-
 Homo sapiens 4-methylumbelliferone + alpha-L-iduronic acid
-
 ?
3.2.1.76 additional information involved in the degradation of dermatan sulfate and heparan sulfate, deficiency in alpha-L-iduronidase causes mucopolysaccharidosis type I Homo sapiens ?
-
 ?

Ki Value [mM]

EC Number Ki Value [mM] Ki Value maximum [mM] Inhibitor Comment Organism Structure
3.2.1.76 1831
-
 NaCl pH 2.8, 37°C, enzyme from healthy individuals Homo sapiens