L-Iduronidase

Known as: Iduronidase, alpha L Idosiduronase, Glycosaminoglycan alpha-L-iduronohydrolase 
An enzyme that hydrolyzes iduronosidic linkages in desulfated dermatan. Deficiency of this enzyme produces Hurler's syndrome. EC 3.2.1.76.
National Institutes of Health

Papers overview

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Highly Cited
2007
Highly Cited
2007
OBJECTIVE Our objective was to evaluate the safety, pharmacokinetics, and efficacy of laronidase in young, severely affected… (More)
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Highly Cited
2004
Highly Cited
2004
OBJECTIVE To confirm the efficacy and safety of recombinant human alpha-L-iduronidase (laronidase) in patients with… (More)
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2004
2004
alpha-L-Iduronidase is a glycosyl hydrolase involved in the sequential degradation of the glycosaminoglycans heparan sulphate and… (More)
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Highly Cited
2003
Highly Cited
2003
Alpha-N-acetylglucosaminidase deficiency (mucopolysaccharidosis IIIB, MPS IIIB) and alpha-l-iduronidase deficiency (MPS I) are… (More)
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Highly Cited
2001
Highly Cited
2001
BACKGROUND Mucopolysaccharidosis I is a lysosomal storage disease caused by a deficiency of the enzyme alpha-L-iduronidase. We… (More)
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Highly Cited
2001
Highly Cited
2001
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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1997
1997
Mucopolysaccharidosis type I (MPS I) is considered to represent the prototypical mucopolysaccharide storage disorder. Although a… (More)
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1992
1992
Mucopolysaccharidosis type I (MPS-I) is an autosomal recessive genetic disease caused by a deficiency of the glycosidase alpha-L… (More)
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1983
1983
A disease discovered in three Plott Hound littermates was found to be associated with a profound and specific deficiency of alpha… (More)
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Highly Cited
1972
Highly Cited
1972
Skin fibroblasts cultured from patients affected with the Hurler or Scheie syndromes (mucopoly-saccharidoses I or V, respectively… (More)
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