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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

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AGT-181
Carbohydrate MetabolismCavity of lysosomeEnzyme GeneHurler-Scheie Syndrome

Papers overview

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Highly Cited
2013
Highly Cited
2013
Enzyme replacement therapy started at birth improves outcome in difficult-to-treat organs in mucopolysaccharidosis I mice.
Highly Cited
2006
Highly Cited
2006
Synthesis of enzymatically active human alpha-L-iduronidase in Arabidopsis cgl (complex glycan-deficient) seeds.
As an initial step to develop plants as systems to produce enzymes for the treatment of lysosomal storage disorders, Arabidopsis… 
Highly Cited
2003
Highly Cited
2003
Treatment of the mouse model of mucopolysaccharidosis I with retrovirally transduced bone marrow.
Highly Cited
1999
Highly Cited
1999
Genetically corrected autologous stem cells engraft, but host immune responses limit their utility in canine alpha-L-iduronidase deficiency.
Canine alpha-L-iduronidase (alpha-ID) deficiency, a model of the human storage disorder mucopolysaccharidosis type I (MPS I), is… 
Highly Cited
1998
Highly Cited
1998
Genotype-phenotype correlations in mucopolysaccharidosis type I using enzyme kinetics, immunoquantification and in vitro turnover studies.
1996
1996
Myoblast gene therapy in canine mucopolysaccharidosis. I: Abrogation by an immune response to alpha-L-iduronidase.
Three dogs with deficiency of the lysosomal enzyme alpha-L-iduronidase were treated by gene replacement therapy targeted at… 
Highly Cited
1983
Highly Cited
1983
The antithrombin-binding sequence in heparin. Identification of an essential 6-O-sulfate group.
Highly Cited
1983
Highly Cited
1983
Coated vesicles from rat liver and calf brain contain lysosomal enzymes bound to mannose 6-phosphate receptors.
Highly Cited
1982
Highly Cited
1982
Is there a mechanism for introducing acid hydrolases into liver lysosomes that is independent of mannose 6-phosphate recognition? Evidence from I-cell disease.
Highly Cited
1976
Highly Cited
1976
Iduronate Sulfatase Activity in Serum, Lymphocytes, and Fibroblasts—Simplified Diagnosis of the Hunter Syndrome
Extract: A previously described assay for iduronate sulfatase has been adapted for use with serum, lymphocytes, and fibroblasts… 
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