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 Maximum [Da] | Comment | Organism |
---|---|---|---|
85000 | - |
x * 85000, SDS-PAGE | Homo sapiens |
480000 | - |
gel filtration and native PAGE | Homo sapiens |
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 | UniProt | Comment | Textmining |
---|---|---|---|
Homo sapiens | - |
- |
- |
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 | Comment | Organism | Textmining |
---|---|---|---|
brain | - |
Homo sapiens | - |
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 | Comment | Organism |
---|---|---|
oligomer | x * 85000, SDS-PAGE | Homo sapiens |
Synonyms | Comment | Organism |
---|---|---|
alpha-L-iduronidase | - |
Homo sapiens |
IDUA | - |
Homo sapiens |
Temperature Optimum [°C] | Temperature Optimum Maximum [°C] | Comment | Organism |
---|---|---|---|
21 | - |
assay at | Homo sapiens |
pH Optimum Minimum | pH Optimum Maximum | Comment | Organism |
---|---|---|---|
3.4 | - |
assay at | Homo sapiens |
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 |