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 | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
lysosome | - |
Homo sapiens | 5764 | - |
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 | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | P35475 | - |
- |
Reaction | Comment | Organism | Reaction ID |
---|---|---|---|
O-(alpha-L-iduronic acid)-(1-4)-D-O-(alpha glucosamine 6-sulfate)-(1-4)-L-O-(alpha-L-iduronic acid)-D-O-2,5-anhydro-mannitol 6-sulfate + H2O = D-O-(alpha glucosamine 6-sulfate)-(1-4)-L-O-(alpha-L-iduronic acid)-D-O-2,5-anhydro-mannitol 6-sulfate + alpha-L-iduronic acid | active site structure, active site residues, overview | Homo sapiens |
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 | Comment | Organism |
---|---|---|
More | 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 | Comment | Organism |
---|---|---|
alpha-L-iduronidase | - |
Homo sapiens |
IDUA | - |
Homo sapiens |
More | the enzyme belongs to the CAZY 39 structural family | Homo sapiens |