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I6034 Sigma-Aldrich

α-L-Iduronidase human

recombinant, expressed in mouse NSO cells

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Properties

Related Categories 3.2.x.x Glycosidases, 3.x.x.x Hydrolases, Biochemicals and Reagents, Enzyme Class Index, Enzymes, Inhibitors, and Substrates More...
Quality Level   200
recombinant   expressed in mouse NSO cells
form   solution
specific activity   ≥7,500 units/μg protein
mol wt   83 kDa
impurities   ≤1.0 EU/μg Endotoxin
shipped in   wet ice
storage temp.   −20°C

Description

General description

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

Application

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

Biochem/physiol Actions

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

Mutation in the α-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.

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

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-α-L-iduronide per minute at pH 3.5 at 25 °C.

Physical form

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

Safety & Documentation

Safety Information

RIDADR 
NONH for all modes of transport
WGK Germany 
WGK 1

Documents

Certificate of Analysis (COA)

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Certificate of Origin (COO)

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Protocols & Articles

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Peer-Reviewed Papers
15

References

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