Iduronidase (EC 3.2.1.76, L-iduronidase, α-L-iduronidase, laronidase), sold as Aldurazyme, is an enzyme with the systematic name glycosaminoglycan α-L-iduronohydrolase.[3][4][5] It catalyses the hydrolysis of unsulfated α-L-iduronosidic linkages in dermatan sulfate.[6]
It is a glycoprotein enzyme found in the lysosomes of cells. It is involved in the degeneration of glycosaminoglycans such as dermatan sulfate and heparan sulfate. The enzyme acts by hydrolyzing the terminal α-L-iduronic acid residues of these molecules, degrading them. The protein is reported as having a mass of approximately 83 kDa.[6]
^ ab"Laronidase (Aldurazyme) Use During Pregnancy". Drugs.com. 11 December 2019. Retrieved 14 April 2020.
^"Aldurazyme 100 U/ml concentrate for solution for infusion - Summary of Product Characteristics (SmPC)". (emc). 2 January 2019. Retrieved 14 April 2020.
^Matalon R, Cifonelli JA, Dorfman A (January 1971). "L-Iduronidase in cultured human fibroblasts and liver". Biochemical and Biophysical Research Communications. 42 (2): 340–5. doi:10.1016/0006-291x(71)90108-2. PMID 4993544.
^Rome LH, Garvin AJ, Neufeld EF (August 1978). "Human kidney α-L-iduronidase: purification and characterization". Archives of Biochemistry and Biophysics. 189 (2): 344–53. doi:10.1016/0003-9861(78)90221-7. PMID 30407.
^Srivastava RM, Hudson N, Seymour FR, Weissman B (1978). "Preparation of (aryl α-L-idopyranosid)uronic acids". Carbohydr. Res. 60 (2): 315–326. doi:10.1016/s0008-6215(78)80038-x.
^ abAldurazyme (Laronidase). BioMarin Pharmaceuticals Inc. FDA website. Retrieved December 6, 2015.
Iduronidase (EC 3.2.1.76, L-iduronidase, α-L-iduronidase, laronidase), sold as Aldurazyme, is an enzyme with the systematic name glycosaminoglycan α-L-iduronohydrolase...
eyes, and heart. The underlying mechanism is a deficiency of alpha-L iduronidase, an enzyme responsible for breaking down GAGs.: 544 Without this enzyme...
enzyme alpha-L-iduronidase. Children born to an MPS I parent carry the defective gene. MPS I H (also called Hurler syndrome or α-L-iduronidase deficiency)...
glycosaminoglycans (or GAGs, or mucopolysaccharides) due to a deficiency of alpha-L iduronidase, an enzyme responsible for the degradation of GAGs in lysosomes. Without...
Scheie syndrome is a disease caused by a deficiency in the enzyme iduronidase, leading to the buildup of glycosaminoglycans (GAGs) in the body. It is...
D., Neufeld, E.F.: Carbohydrate structures of recombinant human a-L-iduronidase secreted by Chinese hamster ovary cells. The Journal of Biological Chemistry...
CAZY GH_39 comprises enzymes with several known activities; alpha-L-iduronidase (EC 3.2.1.76); beta-xylosidase (EC 3.2.1.37). The most highly conserved...
disease type I (or Hurler Syndrome), caused by absence of the enzyme α-l-iduronidase (IDUA) leading to accumulation of glycosaminoglycans (GAG) and resulting...
Maroteaux–Lamy syndrome (also called mucopolysaccharidosis type VI) Iduronidase (branded as Aldurazyme), a recombinant protein therapeutic for mucopolysaccharidosis...
sold as Neulasta alpha-galactosidase A: Fabrazyme by Genzyme alpha-L-iduronidase: (rhIDU; laronidase) Aldurazyme by BioMarin Pharmaceutical and Genzyme...
(November 1992). "PCR detection of two RFLPs in exon I of the alpha-L-iduronidase (IDUA) gene". Human Genetics. 90 (3): 327. doi:10.1007/bf00220095. PMID 1362562...
therapeutic benefit. For example, in human cells with defective α-L-iduronidase (IDUA) expression in cells from patients with IDUA-defective Hurler syndrome...
Hopwood JJ, Morris CP (1992). "Structure and sequence of the human alpha-L-iduronidase gene". Genomics. 13 (4): 1311–3. doi:10.1016/0888-7543(92)90053-U. PMID 1505961...
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