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Literature summary for 3.2.1.76 extracted from

  • Rempel, B.P.; Clarke, L.A.; Withers, S.G.
    A homology model for human alpha-L-iduronidase: insights into human disease (2005), Mol. Genet. Metab., 85, 28-37.
    View publication on PubMed
Search for more literature for 3.2.1.76

Protein Variants

Protein Variants Comment Organism
additional information diverse missense mutations of the human enzyme's core, surface, and active site are correlated to the development of the lysosomal storage disorder mucopolysaccharidosis type I, MPSI, structural implications, detailed overview Homo sapiens

Localization

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

Natural Substrates/ Products (Substrates)

Natural Substrates Organism Comment (Nat. Sub.) Natural Products Comment (Nat. Pro.) Rev. Reac.
additional information Homo sapiens enzyme deficiency causes the lysosomal storage disorder mucopolysaccharidosis type I, MPSI ?
-
 ?

Organism

Organism UniProt Comment Textmining
Homo sapiens P35475
-
-

Reaction

Reaction Comment Organism Reaction ID
hydrolysis of unsulfated alpha-L-iduronosidic linkages in dermatan sulfate active site structure, active site residues, overview Homo sapiens
a

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
additional information enzyme deficiency causes the lysosomal storage disorder mucopolysaccharidosis type I, MPSI Homo sapiens ?
-
 ?

Subunits

Subunits Comment Organism
additional information structure determination, PDB code 1Y24, using the crystal structure of the beta-xylosidase from Thermoanaerobacterium saccharolyticum, EC 3.2.1.37, structure modeling Homo sapiens

Synonyms

Synonyms Comment Organism
alpha-L-iduronidase
-
 Homo sapiens
IDUA
-
 Homo sapiens
additional information the enzyme belongs to the CAZY 39 structural family Homo sapiens