• 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...
    17 KB (1,583 words) - 10:00, 1 September 2024
  • Thumbnail for Hurler syndrome
    eyes, and heart. The underlying mechanism is a deficiency of alpha-L iduronidase, an enzyme responsible for breaking down GAGs.: 544  Without this enzyme...
    16 KB (1,783 words) - 19:54, 23 August 2024
  • Thumbnail for Mucopolysaccharidosis type I
    glycosaminoglycans (or GAGs, or mucopolysaccharides) due to a deficiency of alpha-L iduronidase, an enzyme responsible for the degradation of GAGs in lysosomes. Without...
    7 KB (653 words) - 17:50, 27 March 2024
  • Thumbnail for Scheie syndrome
    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...
    6 KB (520 words) - 19:56, 23 August 2024
  • Thumbnail for Mucopolysaccharidosis
    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)...
    27 KB (3,047 words) - 23:21, 13 August 2024
  • (MPS I H, α-L-iduronidase deficiency) Scheie syndrome (MPS I S) Hurler–Scheie syndrome (MPS I H-S) Hunter syndrome (MPS II, iduronidase sulfate deficiency)...
    4 KB (364 words) - 18:31, 9 February 2024
  • Thumbnail for Hurler–Scheie syndrome
    to break down GAGs in their lysosomes due a deficiency of the enzyme iduronidase. All forms of mucopolysaccharidosis type I (MPS I) are a spectrum of...
    3 KB (188 words) - 04:49, 4 March 2024
  • collagen disease Alpha-ketoglutarate dehydrogenase deficiency Alpha-L-iduronidase deficiency Alpha-mannosidosis Alpha-sarcoglycanopathy Alpha-thalassemia...
    24 KB (1,972 words) - 21:28, 16 August 2024
  • Maroteaux–Lamy syndrome (also called mucopolysaccharidosis type VI) Iduronidase (branded as Aldurazyme), a recombinant protein therapeutic for mucopolysaccharidosis...
    19 KB (1,679 words) - 14:00, 30 August 2024
  • D., Neufeld, E.F.: Carbohydrate structures of recombinant human a-L-iduronidase secreted by Chinese hamster ovary cells. The Journal of Biological Chemistry...
    11 KB (1,312 words) - 19:36, 28 January 2024
  • Thumbnail for Glycoside hydrolase family 39
    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...
    4 KB (371 words) - 21:19, 6 August 2023
  • disease type I (or Hurler Syndrome), caused by absence of the enzyme α-l-iduronidase (IDUA) leading to accumulation of glycosaminoglycans (GAG) and resulting...
    7 KB (852 words) - 04:30, 8 July 2024
  • sold as Neulasta alpha-galactosidase A: Fabrazyme by Genzyme alpha-L-iduronidase: (rhIDU; laronidase) Aldurazyme by BioMarin Pharmaceutical and Genzyme...
    22 KB (2,322 words) - 18:33, 9 February 2024
  • Thumbnail for LEAPER gene editing
    therapeutic benefit. For example, in human cells with defective α-L-iduronidase (IDUA) expression in cells from patients with IDUA-defective Hurler syndrome...
    7 KB (783 words) - 12:15, 22 February 2024
  • 4-β-glucosidase EC 3.2.1.75: glucan endo-1,6-β-glucosidase EC 3.2.1.76: L-iduronidase EC 3.2.1.77: mannan 1,2-(1,3)-α-mannosidase EC 3.2.1.78: mannan endo-1...
    119 KB (15,555 words) - 06:24, 19 September 2024
  • Thumbnail for KCNA4
    (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...
    15 KB (1,799 words) - 23:37, 16 July 2024
  • Thumbnail for Interleukin 5 receptor alpha subunit
    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...
    12 KB (1,569 words) - 05:04, 1 January 2024
  • 811.277.450.529 – hyaluronoglucosaminidase MeSH D08.811.277.450.560 – iduronidase MeSH D08.811.277.450.585 – isoamylase MeSH D08.811.277.450.625 – mannosidases...
    158 KB (15,232 words) - 06:41, 8 March 2024