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Literature summary for 3.2.1.76 extracted from

  • Belichenko, P.V.; Dickson, P.I.; Passage, M.; Jungles, S.; Mobley, W.C.; Kakkis, E.D.
    Penetration, diffusion, and uptake of recombinant human alpha-L-iduronidase after intraventricular injection into the rat brain (2005), Mol. Genet. Metab., 86, 141-149.
    View publication on PubMed

Application

Application Comment Organism
medicine the recombinant enzyme is used in enzyme relacement therapy of the Hurler form of mucopolysaccharidosis type I, MPSI, the enzyme is administered intraventricularly in to 31 Sprague-Dawley rat brains showing penetration of the brain and uptak into neurons and glial cells, rat tissue distribution of human recombinant enzyme activity, overview Homo sapiens

Organism

Organism UniProt Comment Textmining
Homo sapiens
-
-
-

Source Tissue

Source Tissue Comment Organism Textmining
commercial preparation purified recombinant enzyme Homo sapiens
-

Substrates and Products (Substrate)

Substrates Comment Substrates Organism Products Comment (Products) Rev. Reac.
4-methylumbelliferyl-alpha-L-iduronide + H2O
-
Homo sapiens 4-methylumbelliferone + alpha-L-iduronic acid
-
?

Synonyms

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

Temperature Optimum [°C]

Temperature Optimum [°C] Temperature Optimum Maximum [°C] Comment Organism
37
-
assay at Homo sapiens

pH Optimum

pH Optimum Minimum pH Optimum Maximum Comment Organism
5.8
-
assay at Homo sapiens