Literature summary for 3.2.1.76 extracted from

Tsukimura, T.; Tajima, Y.; Kawashima, I.; Fukushige, T.; Kanzaki, T.; Kanekura, T.; Ikekita, M.; Sugawara, K.; Suzuki, T.; Togawa, T.; Sakuraba, H.
Uptake of a recombinant human alpha-L-iduronidase (laronidase) by cultured fibroblasts and osteoblasts (2008), Biol. Pharm. Bull., 31, 1691-1695.

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Activating Compound

Activating Compound	Comment	Organism	Structure
additional information	intracellular IDUA activity increases dose-dependently in both MC3T3-E1 and F665 cells. When laronidase is added to the medium at a low concentration (0.10 mg/ml) there is no difference in the uptake between them. Increase in the enzyme activity is always lower in MC3T3-E1 cells than in F665 ones when laronidase is added to the medium at the concentration of 1.0m g/ml or more, thus uptake of laronidase is lower in MC3T3-E1 cells than in F665 cells	Homo sapiens

Application

Application	Comment	Organism
medicine	administration of a high dose of the enzyme or development of a recombinant alpha-L-iduronidase containing many mannose 6-phosphate residues is required for further improvement of enzyme replacement therapy for skeletal disorders caused by mucopolysaccharidosis I	Homo sapiens

Cloned(Commentary)

Cloned (Comment)	Organism
incorporated via mannose 6-phosphate receptors into human fibroblasts from a patient with MPS I (F17) or a healthy subject (F592), into cultured mouse osteoblasts (MC3T3-E1) or cultured mouse fibroblasts (F665)	Homo sapiens

Inhibitors

Inhibitors	Comment	Organism	Structure
additional information	uptake of laronidase by F17 and F592 cells is strongly inhibited in the presence of 5.0 mmol/l mannose 6-phosphate in the culture medium. Uptake of the enzyme by MC3T3-E1 cells is strongly inhibited by the addition of 5.0 mmol/l M6P to the culture medium, but moderately inhibited by the addition of 5.0 mmol/l Man, as in the case of F665 cells	Homo sapiens

Molecular Weight [Da]

Molecular Weight [Da]	Molecular Weight Maximum [Da]	Comment	Organism
69000	-	Western blot analysis, mature component after incorporation into cells	Homo sapiens
76000	-	Western blot analysis, mature component after incorporation into cells	Homo sapiens
83000	-	Western blot analysis, before incorporation into cells	Homo sapiens

Organism

Organism	UniProt	Comment	Textmining
Homo sapiens	-	-	-

Source Tissue

Source Tissue	Comment	Organism	Textmining

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
IDUA	-	Homo sapiens
laronidase	-	Homo sapiens

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Release 2023.1 (January 2023)