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Literature summary for 3.2.1.76 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
Search for more literature for 3.2.1.76

Inhibitors

Inhibitors Comment Organism Structure
CuCl2 strong noncompetitive inhibitor for enzyme from healthy individuals and mucopolysaccharidosis type I patients Homo sapiens
EDTA competitive, equal inhibition of enzyme from healthy individuals and mucopolysaccharidosis type I patients Homo sapiens
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]

KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
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
0.22
-
 4-methylumbelliferyl-alpha-L-iduronide pH 2.8, 37°C, enzyme from healthy individuals Homo sapiens
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
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

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.
additional information Homo sapiens involved in the degradation of dermatan sulfate and heparan sulfate, deficiency in alpha-L-iduronidase causes mucopolysaccharidosis type I ?
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
4-methylumbelliferyl-alpha-L-iduronide + H2O
-
 Homo sapiens 4-methylumbelliferone + alpha-L-iduronic acid
-
 ?
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]

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