3.2.1.76: L-iduronidase
This is an abbreviated version!
For detailed information about L-iduronidase, go to the full flat file.
Word Map on EC 3.2.1.76
-
3.2.1.76
-
mucopolysaccharidosis
-
lysosomal
-
hurler
-
glycosaminoglycans
-
scheie
-
dermatan
-
heparan
-
hurler-scheie
-
hematopoietic
-
corneal
-
medicine
-
mucopolysaccharide
-
intrathecal
-
clouding
-
hepatosplenomegaly
-
iduronate
-
pompe
-
pharmacology
-
enzyme-replacement
-
4-methylumbelliferyl
-
facies
-
mckusick
-
diagnostics
-
dysostosis
-
fabry
-
drug development
-
gaucher
- 3.2.1.76
- mucopolysaccharidosis
- lysosomal
- hurler
- glycosaminoglycans
-
scheie
- dermatan
- heparan
-
hurler-scheie
-
hematopoietic
- corneal
- medicine
-
mucopolysaccharide
-
intrathecal
-
clouding
-
hepatosplenomegaly
-
iduronate
- pompe
- pharmacology
-
enzyme-replacement
-
4-methylumbelliferyl
-
facies
-
mckusick
- diagnostics
-
dysostosis
- fabry
- drug development
- gaucher
Reaction
Synonyms
Aldurazyme, alpha-IDUA, alpha-iduronidase, alpha-L-iduronidase, alpha-L-iduronidase A, glycosaminoglycan alpha-L-iduronohydrolase, hIDUA, IDU, IDUA, iduronidase, laronidase, More, rIDUA_RLT
ECTree
Advanced search results
Inhibitors
Inhibitors on EC 3.2.1.76 - L-iduronidase
Please wait a moment until all data is loaded. This message will disappear when all data is loaded.
(1R,2R,3R,4S,5S,6R)-3,4,5-trihydroxy-7-[8-[4-([[6-([5-[(3aS,4S,6aR)-2-oxohexahydro-1H-thieno[3,4-d]imidazol-4-yl]pentanoyl]amino)hexanoyl]amino]methyl)-1H-1,2,3-triazol-1-yl]octyl]-7-azabicyclo[4.1.0]heptane-2-carboxylic acid
-
(1R,2R,3R,4S,5S,6R)-7-(8-azidooctyl)-3,4,5-trihydroxy-7-azabicyclo[4.1.0]heptane-2-carboxylic acid
irreversible inhibitor
(1R,2S,3S,4R,5S,6R)-7-(8-azidooctyl)-5-(hydroxymethyl)-7-azabicyclo[4.1.0]heptane-2,3,4-triol
-
(5R,6R,7S,8S)-5,6,7,8-tetrahydro-6,7,8-trihydroxytetrazolo[1,2-a]pyridine-5-carboxylate
-
-
(5R,6S,7S,8S)-5,6,7,8-tetrahydro-6,7,8-trihydroxytetrazolo[1,2-a]pyridine-5-carboxylate
-
-
(5S,6R,7S,8S)-5,6,7,8-tetrahydro-6,7,8-trihydroxytetrazolo[1,2-a]pyridine-5-carboxylate
-
-
2-fluoro-alpha-L-idopyranosyluronic acid fluoride
-
reversible, competitive with 4-nitrophenyl-alpha-L-idopyranosiduronate, activity drops down immediately after inhibitor is added
2-[(1E,3E,5E)-5-[1-(6-[[(1-[8-[(1R,2R,3R,4S,5S,6R)-2-carboxy-3,4,5-trihydroxy-7-azabicyclo[4.1.0]heptan-7-yl]octyl]-1H-1,2,3-triazol-4-yl)methyl]amino]-6-oxohexyl)-3,3-dimethyl-1,3-dihydro-2H-indol-2-ylidene]penta-1,3-dien-1-yl]-1,3,3-trimethyl-3H-indol-1-ium
irreversible inhibitor
5-fluoro-alpha-L-idopyranosyluronic acid fluoride
-
reversible, competitive with 4-methylumbelliferyl-alpha-L-iduronide, activity drops down immediately after inhibitor is added
EDTA
-
competitive, equal inhibition of enzyme from healthy individuals and mucopolysaccharidosis type I patients
[(2S,3R,4R)-3,4-dihydroxy-5-oxotetrahydrofuran-2-yl](hydroxy)acetic acid
-
competitive inhibitor
CuCl2
-
strong noncompetitive inhibitor for enzyme from healthy individuals and mucopolysaccharidosis type I patients
NaCl
-
healthy individuals and 40% of heterocygotic mucopolysaccharidosis type I patients, activating for 60% of heterocygotic mucopolysaccharidosis type I patients
-
not inhibited by 6-sodium 5-amino-5-deoxy-D-glucarate-1,5-lactam, 6-sodium 5-amino-5-deoxy-D-galactarate-1,5-lactam, 6-sodium 5-amino-5-deoxy-L-altrarate-1,5-lactam, and 4-amino-4,5-dideoxy-L-arabinaro-1,4-lactam
-
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
-
additional information
-
deglycosylation of the enzyme with endoglycosidase H, but not peptide-N-glycosidase F, reduces the enzyme's activity
-