Description
This enzyme catalyses the hydrolysis of unsulfated α-L-iduronosidic linkages in dermatan sulfate. In lysosomal degradation process α-L-Iduronidase plays a crucial role. It hydrolyzes the non-reducing terminal α-L-iduronic acid residues in GAGs, including dermatan sulfate and heparan sulfate. It is involved in the degeneration of glycosaminoglycans such as dermatan sulfate and heparan sulfate. It is found in the lysosomes of cells.
Abbr
IDUA, Recombinant (Human)
Applications
α-L-Iduronidase may be used for leukocyte assay in the study of a-L-Iduronidase deficiency in new born.
Form
Supplied as a solution in 40 mM sodium acetate , 400 mM NaCl and 20% (v/v) glycerol, pH 5.0.
Enzyme Commission Number
EC 3.2.1.76
Activity
>7,500 units/μg protein
Unit Definition
One unit will produce 1 picomole of 4-methylumbelliferone from 4-methylumbelliferyl-α-L-iduronide per minute at pH 3.5 at 25 °C.
Synonyms
Iduronidase; EC 3.2.1.76; L-iduronidase; alpha-L-iduronidase); glycosaminoglycan alpha-L-iduronohydrolase; IDUA; α-L-Iduronidase
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