alpha-L-Iduronidase human, recombinant, expressed in mouse NSO cells

Stock Code: 3579737
Manufacturer Part No: I6034-10UG

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Descriptions

Application

?-L-Iduronidase may be used for leukocyte assay in the study of a-L-Iduronidase deficiency in new born.

Biochem/physiol Actions

Catalyzes the hydrolysis of unsulfated α-L-iduronosidic linkages in dermatan sulfate

Mutation in the ?-L-Iduronidase is implicated in Mucopolysaccharidosis I (MPS I) . This enzyme defect leades to accumulation of dermatan and heparan sulfate . MPS I pathophysiology is accompanied with deformation of the skull, mental retardation and hernias.

In lysosomal degradation process ?-L-Iduronidase plays a crucial role. It hydrolyzes the non-reducing terminal ?-L-iduronic acid residues in glycosaminoglycans (GAGs), including dermatan sulfate and heparan sulfate.

General description

?-L-Iduronidase (IDUA) is mapped to human chromosome 4p16.3. The mature IDUA protein is glycosylated and comprises triosephosphate isomerase (TIM) barrel domain, ?-sandwich, helix-loop-helix region and an immunoglobin-like domain. ?-L-Iduronidase is classified under glycoside hydrolase (GH) family 39.

Physical form

Supplied as a solution in 40 mM sodium acetate , 400 mM NaCl and 20% (v/v) glycerol, pH 5.0

Physical properties

Expressed as C-terminal histine-tagged protein (residues 1-653) with a caluclated molecular mass of 71 kDa migrating at ~83 kDa under SDS-PAGE reducing conditions.

Unit Definition

One unit will produce 1 picomole of 4-methylumbelliferone from 4-methylumbelliferyl-A-L-iduronide per minute at pH 3.5 at 25 °C.