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Literature summary for 3.2.1.76 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
Search for more literature for 3.2.1.76

Application

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

Molecular Weight [Da]

Molecular Weight [Da] Molecular Weight Maximum [Da] Comment Organism
85000
-
 x * 85000, SDS-PAGE Homo sapiens
480000
-
 gel filtration and native PAGE Homo sapiens

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
additional information Homo sapiens the enzyme hydrolyzes terminal iduronic acid residues on glycosaminoglycans ?
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Posttranslational Modification

Posttranslational Modification Comment Organism
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
phosphoprotein phosphorylations on S59 and S482, phosphatase treatment Homo sapiens
side-chain modification histidine methylation at H572 Homo sapiens

Source Tissue

Source Tissue Comment Organism Textmining
brain
-
 Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
4-methylumbelliferyl-alpha-L-iduronide + H2O
-
 Homo sapiens 4-methylumbelliferol + alpha-L-iduronic acid
-
 ?
additional information the enzyme hydrolyzes terminal iduronic acid residues on glycosaminoglycans Homo sapiens ?
-
 ?

Subunits

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

Synonyms

Synonyms Comment Organism
alpha-L-iduronidase
-
 Homo sapiens
IDUA
-
 Homo sapiens

Temperature Optimum [°C]

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

pH Optimum

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

General Information

General Information Comment Organism
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