FAQ

L-iduronidase activity

Known as: alpha-L-iduronidase activity, glycosaminoglycan alpha-L-iduronohydrolase activity 
Catalysis of the hydrolysis of alpha-L-iduronosidic linkages in dermatan sulfate. [EC:3.2.1.76]
National Institutes of Health

Topic mentions per year

Topic mentions per year

1972-2012
02419722012

Papers overview

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Highly Cited
2004
Highly Cited
2004
Cord-blood transplants from unrelated donors in patients with Hurler's syndrome.
BACKGROUND Hurler's syndrome (the most severe form of mucopolysaccharidosis type I) causes progressive deterioration of the… (More)
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2002
2002
Mucopolysaccharidosis type I: Identification and characterization of mutations affecting alpha-L-iduronidase activity.
Mucopolysaccharidosis type I (MPS I) is caused by a deficiency of the lysosomal enzyme alpha-L-iduronidase (IDUA). MPS I covers a… (More)
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2001
2001
Gentamicin-mediated suppression of Hurler syndrome stop mutations restores a low level of alpha-L-iduronidase activity and reduces lysosomal glycosaminoglycan accumulation.
Hurler syndrome is the most severe form of a lysosomal storage disease caused by loss of the enzyme alpha-L-iduronidase (encoded… (More)
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Highly Cited
2001
Highly Cited
2001
Enzyme-replacement therapy in mucopolysaccharidosis I.
BACKGROUND Mucopolysaccharidosis I is a lysosomal storage disease caused by a deficiency of the enzyme alpha-L-iduronidase. We… (More)
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1999
1999
Mucopolysaccharidosis type I: characterization of novel mutations affecting alpha-L-iduronidase activity.
alpha-L-Iduronidase (IDUA) deficiency (mucopolysaccharidosis type I, MPS I) involves a broad spectrum of clinical severity… (More)
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1992
1992
Immunoquantification and enzyme kinetics of alpha-L-iduronidase in cultured fibroblasts from normal controls and mucopolysaccharidosis type I patients.
alpha-L-Iduronidase activity is deficient in mucopolysaccharidosis type I (MPS I; Hurler syndrome, Scheie syndrome) patients and… (More)
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1980
1980
Fluorometric measurement of alpha-L-iduronidase activity using 4-methylumbelliferyl-alpha-L-iduronide.
Using 4-methylumbelliferyl-alpha-L-iduronide as a substrate, alpha-L-iduronidase activity was measured in leukocytes and in… (More)
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1979
1979
A fluorometric assay using 4-methylumbelliferyl alpha-L-iduronide for the estimation of alpha-L-iduronidase activity and the detection of Hurler and Scheie syndromes.
Incubation of 4-methylumbelliferyl alpha-L-iduronide with whole cell homogenates prepared from cultured skin fibroblasts and… (More)
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1978
1978
Fluorometric measurement of urinary alpha-L-iduronidase activity.
A fluorogenic substrate for alpha-L-iduronidase, 4-methylumbelliferyl alpha-L-iduronide, has been newly synthesized and the… (More)
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1972
1972
The defect in the Hurler and Scheie syndromes: deficiency of -L-iduronidase.
Skin fibroblasts cultured from patients affected with the Hurler or Scheie syndromes (mucopoly-saccharidoses I or V, respectively… (More)
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