Iduronidase
| Iduronidase | ||
|---|---|---|
| Properties of human protein | ||
| Mass / length primary structure | 626 amino acids | |
| Secondary to quaternary structure | Monomer | |
| Identifier | ||
| Gene name | IDUA | |
| External IDs | ||
| Drug information | ||
| ATC code | A16 AB05 | |
| DrugBank | DB00090 | |
| Drug class | Enzyme replacement | |
| Enzyme classification | ||
| EC, category | 3.2.1.76 , glycosidase | |
| Response type | hydrolysis | |
| Substrate | Dermatan sulfate, heparan sulfate | |
| Occurrence | ||
| Homology family | Iduronidase | |
| Parent taxon | Eukaryotes | |
Iduronidase , more precisely α-L-iduronidase (IDUA), is the name of the enzyme that in many mammals splits L-iduronate from dermatan sulfate and heparan sulfate , the main components of fibers and cartilage tissue in the body. It is therefore an indispensable part of the reaction pathway that breaks down these substances in the body. It is made in the lysosomes of all cells. Mutations at IDUA - gene can deficiency of this enzyme and so when people have different types of mucopolysaccharidosis cause.
Catalyzed reaction
When breaking down dermatan sulfate
IdoAβ1-3GalNAc (4S) β1-4GlcAβ1-3GalNAc (4S) + H 2 O → 3GalNAc (4S) β1-4GlcAβ1-3GalNAc (4S) + L-iduronate
When breaking down heparan sulfate
IdoAβ1-4GlcN (2S) α1-4GlcA (2S) β1-4GlcNAc (6S) α1-4GlcA
+ H 2 O → 4GlcN (2S) α1-4GlcA (2S) β1-4GlcNAc (6S) α1-4GlcA + L-iduronate
Pharmaceutical preparations
The intake of iduronidase can counteract certain clinical pictures that are associated with a corresponding enzyme deficiency.
Monopreparations
Aldurazyme ® , manufacturer: Genzyme
Web links
- Public Assessment Report (EPAR) of the European Medicines Agency (EMA) on: Iduronidase
