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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...
recombinant   expressed in mouse NSO cells
activity   ≥7,500 units/μg protein
impurities   ≤1.0 EU/μg Endotoxin
shipped in   wet ice
storage temp.   −20°C

Description

General description

α-L-Iduronidase is an enzyme encoded by IDUA gene; participate in lysosomal degradation of glycosaminoglycans. In human, mutation of gene encoding α-L-Iduronidase causes lysosomal storage diseases (mucopolysaccharidosis type I).

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 GAGs, including dermatan sulfate and heparan sulfate.

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 
1

Documents

Certificate of Analysis (COA)

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Certificate of Origin (COO)

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Protocols & Articles

Related Content

Enzymes & Proteins

Application Index | Enzyme Index | Substrate Index | Inhibitor Index | Cofactor Index | Lectin Index
Keywords: Cell signaling, Diagnostic, Drug discovery, Metabolomics, Molecular biology

Peer-Reviewed Papers
15

References

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