Iduronidase (EC 3.2.1.76, L-iduronidase, α-L-iduronidase, laronidase), sold as Aldurazyme, is an enzyme with the systematic ... Iduronidase at the U.S. National Library of Medicine Medical Subject Headings (MeSH) Portals: Biology Medicine (Webarchive ... Aldurazyme is the name of the commercialized variant of the enzyme iduronidase, which hydrolyzes the α-L-iduronic acid residues ... The defective α-L-iduronidase results in an accumulation of heparan and dermatan sulfate within phagocytes, endothelium, smooth ...
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Iduronidase / therapeutic use* * Male * Middle Aged * Mucopolysaccharidoses / enzymology* * Mucopolysaccharidoses / ...
IDUA: alpha-L-iduronidase. *IFIH1: interferon induced with helicase C domain 1 ...
alpha-L-iduronidase premature stop codons and potential read-through in mucopolysaccharidosis type I patients. J Mol Biol. 2004 ... 4p16.3 (Hurler syndrome, Scheie syndrome): The activity of alpha-L-iduronidase is decreased in Hurler syndrome and Scheie ... Gentamicin-mediated suppression of Hurler syndrome stop mutations restores a low level of alpha-L-iduronidase activity and ... making a total of 15 different mutations that can cause premature stop codons in the alpha-L-iduronidase gene (IDUA), and the ...
alpha-L-iduronidase premature stop codons and potential read-through in mucopolysaccharidosis type I patients. J Mol Biol. 2004 ... Gentamicin-mediated suppression of Hurler syndrome stop mutations restores a low level of alpha-L-iduronidase activity and ... multinational study of recombinant human alpha-L-iduronidase (laronidase). J Pediatr. 2004 May. 144(5):581-8. [QxMD MEDLINE ...
alpha-L-iduronidase. involved_in. ISO. MGI:2176236,MGI:2651471 (MGI:3605936,PMID:15979918), (MGI:7310026,PMID:25854773). RGD. ... alpha-L-iduronidase. involved_in. acts_upstream_of_or_within. ISO. (PMID:24036510). MGI:2176236,MGI:4415188 (MGI:4414887,PMID: ... alpha-L-iduronidase. acts_upstream_of_or_within. ISO. MGI:2176236,MGI:4415188 (MGI:4397742,PMID:19834056), (MGI:4414887,PMID: ...
Lenti ORF clone of Human iduronidase, alpha-L- (IDUA), Myc-DDK-tagged. * * 10 ug. 10 ug ... Lenti ORF clone of Human iduronidase, alpha-L- (IDUA), Myc-DDK-tagged. * * 10 ug. 10 ug ... Lenti ORF clone of Human iduronidase, alpha-L- (IDUA), mGFP tagged. * * 10 ug. 10 ug ... Lenti ORF clone of Human iduronidase, alpha-L- (IDUA), mGFP tagged. * * 10 ug. 10 ug ...
alpha-L-iduronidase premature stop codons and potential read-through in mucopolysaccharidosis type I patients. J Mol Biol. 2004 ... 4p16.3 (Hurler syndrome, Scheie syndrome): The activity of alpha-L-iduronidase is decreased in Hurler syndrome and Scheie ... Gentamicin-mediated suppression of Hurler syndrome stop mutations restores a low level of alpha-L-iduronidase activity and ... making a total of 15 different mutations that can cause premature stop codons in the alpha-L-iduronidase gene (IDUA), and the ...
iduronidase, alpha-L. Tssr52446. 5. 108660744 to 108660778 34. -. TSS region. transcription start site region 52446. ...
In MPS I the enzyme that is lacking is called α-L-iduronidase. Since patients with MPS I cannot break GAGs down properly, they ...
Safe and Sustained Expression of Human Iduronidase After Intrathecal Administration of Adeno-Associated Virus Serotype 9 in ...
Alpha-L-iduronidase deficiency. *Aluminium (dialysis related) toxicity. *Aminomethyltransferase deficiency. *Aminophylline. * ...
The drug candidate is a recombinant blood brain barrier penetrating acid alpha-L iduronidase. It is administered by intravenous ...
Methods and compositions for increasing alpha-l-iduronidase activity in the cns ... Methods and compositions for increasing alpha-l-iduronidase activity in the cns ...
Genetic studies showed lower amounts of alpha L-Iduronidase (IDUA) in the glaucomatous outflow pathway. IDUA is a lysosomal ...
The assay uses 4-methylumbelliferyl substrates to measure the activities of acid sphingomyelinase, alpha-iduronidase, beta- ...
It is also targeting Mucopolysaccharidosis type I (MPS I), or Hurler syndrome, by delivering the α-L-iduronidase gene to the ...
In vivo delivery of human alpha-L-iduronidase in mice implanted with neo-organs. Hum Gene Ther. 1995 Sep; 6(9):1153-9. ...
Therapeutic Candidate or Device Autologous blood stem cells edited to restore iduronidase expression Indication Severe ...
Mucopolysaccharidosis Type 1 (MPSI) is a lysosomal storage disease caused by deficient activity of the alpha-L-iduronidase ...
Mucopolysaccharidosis I (MPS-I). Caused by a failure to synthesize an enzyme (α-L-iduronidase) needed to break down ...
L-iduronidase, xiphoid propecia dyspareunia: infiltrate, kerb. Perform phk.hsty.matematik19.info.pkc.cd hypothetical ...
Aldurazyme Reduction of symptoms in sufferers with 10/20/2006 L-iduronidase eight/25/1999 Genzyme Japan K [url=http://www. ...
Morquio syndrome (mucopolysaccharidosis type IV) is a member of a group of inherited metabolic disorders collectively termed mucopolysaccharidoses (MPSs). The MPSs are caused by a deficiency of lysosomal enzymes required for the degradation of mucopolysaccharides or glycosaminoglycans (GAGs).
Morquio syndrome (mucopolysaccharidosis type IV) is a member of a group of inherited metabolic disorders collectively termed mucopolysaccharidoses (MPSs). The MPSs are caused by a deficiency of lysosomal enzymes required for the degradation of mucopolysaccharides or glycosaminoglycans (GAGs).
iduronidase, alpha-L-. Felis catus. domestic cat. protein-coding. ITGA2B. integrin, alpha 2b (platelet glycoprotein IIb of IIb/ ...
Toxicology study of intra-cisterna magna adeno-associated virus 9 expressing human Alpha-L-iduronidase in rhesus macaques. Mol ... Safe and sustained expression of human iduronidase after intrathecal administration of adeno-associated virus serotype 9 in ... a pre-clinical study investigating gene transfer following IT delivery of an AAV9 vector expressing α-1-iduronidase (IDUA) to ...