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Iduronidase, a-L-, Recombinant, Human (a-L-Iduronidase, IDUA)

Cat no: I0706-94

Iduronidase, a-L-, Recombinant, Human (a-L-Iduronidase, IDUA)

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. Mutations in IDUA that result in enzymatic deficiency lead to the autosomal recessive disease mucopolysaccharidosis type I (MPS I) (1). MPS I causes progressive cellular, tissue and organ damage, and several clinical studies using enzyme replacement therapy have shown promising benefits (2).\n\nThe purified rhIDUA has an N-terminal sequence of T26EAPHLVQVD. The 638 amino acid residue rhIDUA predicts a molecular mass of 71kD, and migrates as an ~83 kD protein by SDS-PAGE under reducing conditions.\n\nRecombinant protein corresponding to Ala26-Pro653 (Ala26Thr) with a C-terminal 10-His tag expressed in NSO cells (AAA81589).\n\nMolecular Mass: \nThe purified rhIDUA has an N-terminal sequence of T26EAPHLVQVD. The 638 amino acid residue rhIDUA predicts a molecular mass of 71kD, and migrates as an approximately 83kD protein by SDS-PAGE under reducing conditions.\n\nActivity: \nMeasured by its ability to cleave a fluorogenic substrate, 4-Methylumbelliferyl alpha-L-iduronide. \n\nStorage and Stability: \nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for 6 months at -20 degrees C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

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SPECIFICATIONS

Catalog Number

I0706-94

Size

10ug

Form

Supplied as a liquid in 50mM sodium acetate, 500mM sodium chloride, pH 5.0.

Purity

~ 95%, as determined by SDS-PAGE and visualized by silver stain. Endotoxin: (same/less than) 1EU/ug (LAL)

References

1. Scott, H.S. et al., 1995, Hum. Mutat. 6:288. 2. Wraith, J.E. 2005, Expert Opin. Pharmacother. 6:489.

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SUPPLIER INFO

United States Biological
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