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 | 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 | Comment | Organism | GeneOntology No. | Textmining |
---|---|---|---|---|
lysosome | - |
Homo sapiens | 5764 | - |
lysosome | - |
Felis catus | 5764 | - |
Organism | UniProt | Comment | Textmining |
---|---|---|---|
Felis catus | - |
- |
- |
Homo sapiens | - |
- |
- |
Source Tissue | Comment | Organism | Textmining |
---|---|---|---|
brain | - |
Felis catus | - |
Synonyms | Comment | Organism |
---|---|---|
alpha-L-iduronidase | - |
Homo sapiens |
alpha-L-iduronidase | - |
Felis catus |
IDU | - |
Homo sapiens |
IDU | - |
Felis catus |
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 | 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 |