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
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3.2.1.76
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mucopolysaccharidosis
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lysosomal
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hurler
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glycosaminoglycans
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scheie
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dermatan
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heparan
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hurler-scheie
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hematopoietic
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corneal
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medicine
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mucopolysaccharide
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intrathecal
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clouding
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hepatosplenomegaly
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iduronate
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pompe
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pharmacology
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enzyme-replacement
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4-methylumbelliferyl
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facies
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mckusick
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diagnostics
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dysostosis
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fabry
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drug development
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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
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Cloned
Cloned on EC 3.2.1.76 - L-iduronidase
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commercially available recombinant human iduronidase (Aldurazyme) produced in Chinese-hamster-ovary cells
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expression in Cos-1 cells
expression of IDUA with and without a C-terminal endoplasmic reticulum-retention sequence SEKDEL, in seeds of Brassica napus and Nicotiana tabacum under the control of regulatory 5'-, signal-peptide-encoding-, and 3'-sequences from the arcelin 5-I gene of Phaseolus vulgaris
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expression of wild-type enzyme and enzyme in fusion with the human receptor-associated protein, RAP, in LRP1-null CHO-K1 cells
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gene IDUA, screening and genotyping of nonsense, 4 missense, 1 deletion, and 2 splice site intron mutations in 10 MPS I patients, development and evaluation of a dHPLC screening method, overview
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genotyping of different mucopolysaccharidosis type I patient cell lines, overview
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IDUA cloning in Escherichia coli strain BL21, and overexpression in transgenic Nicotiana tabacum BY-2 cells, establishing and evaluation of the expression system, overview. A plant signal peptide is essential for proper expression and secretion of the glycosylated recombinant hIDUA into the cultured media of transgenic BY-2 cells, transfection by Agrobacterium tumefaciens, strain LBA4404
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IDUA gene, determination of mutant sequences from mucopolysaccharidosis type I enzymes, genotyping, expression of wild-type and mutant enzymes in CHO cells
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)
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