α-L-Iduronidase human
SIGMA/I6034 - recombinant, expressed in mouse NSO cells
Synonym: IDUA
Product Type: Chemical
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| form | solution |
| impurities | ≤1.0 EU/μg Endotoxin |
| mol wt | 83 kDa |
| Quality Level | 200  |
| recombinant | expressed in mouse NSO cells |
| shipped in | wet ice |
| specific activity | ≥7,500 units/μg protein |
| storage temp. | −20°C |
| Application: | α-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- |
| 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. |
| Biochem/physiol Actions: | 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. |
| General description: | 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. |
| General description: | α- |
| Other Notes: | One unit will produce 1 picomole of 4-methylumbelliferone from 4-methylumbelliferyl-α-L- |
| Physical form: | Supplied as a solution in 40 mM sodium acetate , 400 mM NaCl and 20% (v/v) glycerol, pH 5.0 |
| RIDADR | NONH for all modes of transport |
| WGK Germany | WGK 1 |
| Flash Point(F) | Not applicable |
| Flash Point(C) | Not applicable |
| activity | specific activity: ≥7,500 units/μg protein |
| Storage Temp. | −20°C |
| Enzyme Commission (EC) Number | 3.2.1.76 ( BRENDA  | IUBMB ) |
| UNSPSC | 12352204 |