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Literature summary for 3.2.1.76 extracted from

  • Mandelli, J.; Wajner, A.; Pires, R.F.; Giugliani, R.; Coelho, J.C.
    Detection of mucopolysaccharidosis type I heterozygotes based on the biochemical characteristics of leukocyte alpha-L-iduronidase (2002), Arch. Med. Res., 33, 20-24.
    View publication on PubMed

KM Value [mM]

KM Value [mM] KM Value Maximum [mM] Substrate Comment Organism Structure
0.38
-
4-methylumbelliferyl-alpha-L-iduronide 37°C, enzyme from mucopolysaccharidosis patients group II Homo sapiens
0.49
-
4-methylumbelliferyl-alpha-L-iduronide 37°C, enzyme from mucopolysaccharidosis patients group I Homo sapiens
0.6
-
4-methylumbelliferyl-alpha-L-iduronide 37°C, enzyme from healthy individuals Homo sapiens

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
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
leukocyte
-
Homo sapiens
-

Specific Activity [micromol/min/mg]

Specific Activity Minimum [µmol/min/mg] Specific Activity Maximum [µmol/min/mg] Comment Organism
additional information
-
specific activity in healthy individuals was found to be higher than specific activity in mucopolysaccharidosis patients group I but similar with mucopolysaccharidosis patients group II 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 ?
-
?

Temperature Stability [°C]

Temperature Stability Minimum [°C] Temperature Stability Maximum [°C] Comment Organism
50
-
enzyme from mucopolysaccharidosis patients is more stable than enzyme from healthy individuals Homo sapiens

pH Optimum

pH Optimum Minimum pH Optimum Maximum Comment Organism
2.7
-
-
Homo sapiens