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Literature summary extracted from

  • Brooks, D.A.; Fabrega, S.; Hein, L.K.; Parkinson, E.J.; Durand, P.; Yogalingam, G.; Matte, U.; Giugliani, R.; Dasvarma, A.; Eslahpazire, J.; Henrissat, B.; Mornon, J.P.; Hopwood, J.J.; Lehn, P.
    Glycosidase active site mutations in human alpha-L-iduronidase (2001), Glycobiology, 11, 741-750.
    View publication on PubMed

Cloned(Commentary)

EC Number Cloned (Comment) Organism
3.2.1.76 expressed in CHO-K1 cells Homo sapiens

Protein Variants

EC Number Protein Variants Comment Organism
3.2.1.76 E182A catalytically inactive Homo sapiens
3.2.1.76 E182K mutation found in patients with mucopolysaccharidosis type I, catalytically inactive Homo sapiens
3.2.1.76 E299A catalytically inactive Homo sapiens

Localization

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

Molecular Weight [Da]

EC Number Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
3.2.1.76 74000
-
 wild type enzyme, SDS-PAGE, intracellulary processed to form fragments of 69000 Da and 65000 Da Homo sapiens

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
-
-
-

Posttranslational Modification

EC Number Posttranslational Modification Comment Organism
3.2.1.76 side-chain modification N-linked oligosaccharides 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 ?
-
 ?