Any feedback?
Please rate this page
(literature.php)
(0/150)

BRENDA support

Literature summary extracted from

  • Jung, G.; Pabst, M.; Neumann, L.; Berger, A.; Lubec, G.
    Characterization of alpha-L-iduronidase (Aldurazyme) and its complexes (2013), J. Proteomics, 80, 26-33.
    View publication on PubMed

Application

EC Number Application Comment Organism
3.2.1.76 medicine alpha-L-iduronidase is used in enzyme replacement therapy approved for mucopolysaccharidosis type I treatment Homo sapiens

Molecular Weight [Da]

EC Number Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
3.2.1.76 85000
-
 x * 85000, SDS-PAGE Homo sapiens
3.2.1.76 480000
-
 gel filtration and native PAGE 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 the enzyme hydrolyzes terminal iduronic acid residues on glycosaminoglycans ?
-
 ?

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 glycoprotein N-glycosylation, deglycoslyation b peptide N-glycosidase F from Flavobacterium meningosepticum and neuraminidase from Clostridium perfringens. The N-glycan pools consists of oligomannosidic glycans (mainly Man7, Man8, Man9 as well as Man9 + Glc), complex type glycans with up to three antennae. Sialylation as well as fucosylation are observed on almost all complex type glycans Homo sapiens
3.2.1.76 phosphoprotein phosphorylations on S59 and S482, phosphatase treatment Homo sapiens
3.2.1.76 side-chain modification histidine methylation at H572 Homo sapiens

Source Tissue

EC Number Source Tissue Comment Organism Textmining
3.2.1.76 brain
-
 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-methylumbelliferol + alpha-L-iduronic acid
-
 ?
3.2.1.76 additional information the enzyme hydrolyzes terminal iduronic acid residues on glycosaminoglycans Homo sapiens ?
-
 ?

Subunits

EC Number Subunits Comment Organism
3.2.1.76 oligomer x * 85000, SDS-PAGE Homo sapiens

Synonyms

EC Number Synonyms Comment Organism
3.2.1.76 alpha-L-iduronidase
-
 Homo sapiens
3.2.1.76 IDUA
-
 Homo sapiens

Temperature Optimum [°C]

EC Number Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
3.2.1.76 21
-
 assay at Homo sapiens

pH Optimum

EC Number pH Optimum Minimum pH Optimum Maximum Comment Organism
3.2.1.76 3.4
-
 assay at Homo sapiens

General Information

EC Number General Information Comment Organism
3.2.1.76 malfunction deficiency of the enzyme leads to progressive accumulation of glycosaminoglycans, dermatan and heparan sulfate, in all organs and tissues, mucopolysccharidosis I is a lysosomal storage disorder that results from deficiency in the lysosomal enzyme alpha-L-iduronidase. Infusions of intravenous enzyme improves hepatosplenomegaly, pulmonary function, ambulation, jointmobility, cardiac function, and cognitive and neuroradiological progress Homo sapiens