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Literature summary for 3.2.1.76 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
Search for more literature for 3.2.1.76

Cloned(Commentary)

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

Protein Variants

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

Localization

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

Molecular Weight [Da]

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

Posttranslational Modification

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