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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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2007
2007
Pre‐stem cell transplantation enzyme replacement therapy in Hurler syndrome does not lead to significant antibody formation or delayed recovery of the endogenous enzyme post‐transplant: A case report
Abstract:  Combined enzyme replacement therapy (ERT) and stem cell transplant (SCT) were done for a two year old boy with severe… 
Review
1985
Review
1985
The clinical spectrum of alpha-L-iduronidase deficiency.
We present five patients with alpha-L-iduronidase deficiency who do not have the typical Hurler or Scheie phenotypes; they are… 
1985
1985
Fluorometric assay of alpha-L-iduronidase in serum for detection of affected and carrier animals in a canine model of mucopolysaccharidosis I.
We investigated measuring serum alpha-L-iduronidase (EC 3.2.1.76) by a sensitive fluorometric assay in 28 members of a canine… 
1984
1984
Regional assignment of the structural gene for human alpha-L-iduronidase.
The structural gene encoding human alpha-L-iduronidase has been assigned to chromosome 22 by using immunologic, electrophoretic… 
1982
1982
Diagnostic enzymology of alpha-L-iduronidase with special reference to a sulphated disaccharide derived from heparin.
1. Iduronosyl anhydro[1-3H] mannitol 6-sulphate (IMs), iduronosyl anhydro [1 3H] mannitol, phenyl iduronide (PhI) and 4… 
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… 
1977
1977
alpha-L-Iduronidase activity in established lymphoblastoid cells from patients with Hurler and Scheie syndromes transformed by Epstein-Barr virus.
alpha-L-Iduronidase activity was determined in established lymphoblastoid cells, which were transformed in vitro by Epstein-Barr… 
Review
1976
Review
1976
The mucopolysaccharidoses and mucolipidoses.
The mucopolysaccharidoses and mucolipidoses are recessively inherited lysosomal storage diseases. Each of the disorders can now… 
1976
1976
Atypical Hurler syndrome without alpha-L-iduronidase deficiency.
Three atypical patients with clinical and laboratory findings of Hurler syndrome, but without alpha-L-iduronidase deficiency, are… 
1976
1976
Leucocyte values of alpha-L-iduronidase activity in mucopolysaccharidosis I.
Assay of alpha-L-iduronidase in peripheral leucocytes is a rapid and simple diagnostic aid in mucopolysaccharidosis I. The mean… 
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