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Literature summary for 3.2.1.76 extracted from

  • Vite, C.H.; Wang, P.; Patel, R.T.; Walton, R.M.; Walkley, S.U.; Sellers, R.S.; Ellinwood, N.M.; Cheng, A.S.; White, J.T.; O’Neill, C.A.; Haskins, M.
    Biodistribution and pharmacodynamics of recombinant human alpha-L-iduronidase (rhIDU) in mucopolysaccharidosis type I-affected cats following multiple intrathecal administrations (2011), Mol. Genet. Metab., 103, 268-274.
    View publication on PubMedView publication on EuropePMC
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Application

Application Comment Organism
medicine intrathecal administration of recombinant human IDU, with potential dosing every 2-3 months, may provide benefit for the treatment of central nervous system disease in mucopolysaccharidosis type I patients Homo sapiens
medicine intrathecal administration of recombinant human IDU, with potential dosing every 2-3 months, may provide benefit for the treatment of central nervous system disease in mucopolysaccharidosis type I patients Felis catus

General Stability

General Stability Organism
the lysosomal (or tissue) half-life of recombinant human IDU appears to be approximately 6-7 days because a 3fold higher than normal enzyme level (300%) elevation is reduced to about 20% over a period of 28 days Homo sapiens

Localization

Localization Comment Organism GeneOntology No. Textmining
lysosome
-
 Homo sapiens 5764
-
lysosome
-
 Felis catus 5764
-

Organism

Organism UniProt Comment Textmining
Felis catus
-
-
-
Homo sapiens
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
brain
-
 Felis catus
-

Synonyms

Synonyms Comment Organism
alpha-L-iduronidase
-
 Homo sapiens
alpha-L-iduronidase
-
 Felis catus
IDU
-
 Homo sapiens
IDU
-
 Felis catus

Expression

Organism Comment Expression
Felis catus following three repeat intrathecal administrations of 0.1 mg/kg recombinant human alpha-L-iduronidase or placebo on days 1, 4 or 5, and 9, two days after the final intrathecal injection, the mean tissue alpha-L-iduronidase activity in the brains of the two treated animals are approximately 3times higher than the activity found in normal cat brains and remains higher than untreated mucopolysaccharidosis type I brain at 1 month before returning to near-baseline levels after 2 months up

General Information

General Information Comment Organism
malfunction mucopolysaccharidosis type I is caused by a deficiency in lysosomal alpha-L-iduronidase activity Homo sapiens
malfunction mucopolysaccharidosis type I is caused by a deficiency in lysosomal alpha-L-iduronidase activity Felis catus