|Product Overview:||Recombinant Mouse Idua (Glu17-Ser634) fused with His tag at C-terminal was expressed in NS0.|
|Description:||alpha -L-Iduronidase encoded by the IDUA gene is an important enzyme required for the lysosomal degradation of glycosaminoglycans (GAGS). It hydrolyzes the non-reducing terminal alpha -L-iduronic acid residues in GAGS including dermatan sulfate and heparan sulfate. Mature mouse IDUA shares 80% aa identity with human IDUA. Mutations in IDUA that result in enzymatic deficiency lead to the autosomal recessive disease mucopolysaccharidosis type I (MPS I). MPS I can be classified as three clinical subtypes; Hurler syndrome, Hurler-Scheie syndrome, and Scheie syndrome with decreasing severity, respectively. MPS I causes progressive cellular, tissue and organ damage, and several clinical studies using enzyme replacement therapy show positive results. Recently, the IDUA gene has been linked to osteoporosis.|
|Predicted N Terminal:||Glu17|
|Form:||Supplied as a 0.2 μm filtered solution in Sodium Acetate, NaCl and Glycerol.|
|Bio-activity:||Measured by its ability to cleave a fluorogenic substrate, 4-Methylumbelliferyl alpha -L-iduronide. The specific activity is >7,500 pmol/min/ug.|
|Molecular Mass:||Predicted Molecular Mass: 70 kDa
SDS-PAGE: 83-95 kDa, reducing conditions
|Endotoxin:||<0.1 EU per 1 μg of the protein by the LAL method.|
|Purity:||>95%, by SDS-PAGE visualized with Silver Staining and quantitative densitometry by Coomassie® Blue Staining.|
|Storage:||Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
6 months from date of receipt, -20 to -70 centigrade as supplied.
3 months, -20 to -70 centigrade under sterile conditions after opening.
|Gene Name:||Idua iduronidase, alpha-L- [ Mus musculus ]|
|Synonyms:||IDUA; iduronidase, alpha-L-; alpha-L-iduronidase; 6030426D08;|
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