Iduronidase

  • OMIM: 252800
  • UniProt: P35475
  • MGI: 96418
  • CAS Number: 210589-09-6

Iduronidase (trade name: Aldurazyme ®, manufactured by Genzyme), more α -L- iduronidase ( IDUA ) is the name for the enzyme that cleaves in many mammals, L- iduronate of dermatan sulfate and heparan sulfate, the main components of fibers and cartilage tissue in the body. It is thus indispensable part of the pathway, which degrades these substances in the body. It is formed in the lysosomes of all cells. Mutations in the IDUA gene can cause various types of mucopolysaccharidosis deficiency of this enzyme and so in humans. One way of recovery is then to be administered in addition iduronidase; For this purpose, the active ingredient Laronidase was developed.

Catalyzed reaction

In the degradation of dermatan

IdoAβ1 - 3GalNAc ( 4S) β1 - 3GalNAc 4GlcAβ1 - (4S ) H2O → 3GalNAc ( 4S) β1 - 3GalNAc 4GlcAβ1 - (4S ) L- iduronate

The degradation of heparan sulfate

IdoAβ1 - 4GlcN ( 2S) α1 - 4GlcA ( 2S) β1 - 4GlcNAc ( 6S) α1 - 4GlcA H2O → 4GlcN ( 2S) α1 - 4GlcA ( 2S) β1 - 4GlcNAc ( 6S) α1 - 4GlcA L- iduronate

Human Genome Organisation Mouse Genome Informatics CAS registry number Anatomical Therapeutic Chemical Classification System DrugBank Glycoside hydrolase Hydrolysis
407456
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