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).
The 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 ~83kD protein by SDS-PAGE under reducing conditions.
Recombinant protein corresponding to Ala26-Pro653 (Ala26Thr) with a C-terminal 10-His tag expressed in NSO cells (AAA81589).
Molecular Mass:The 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.
Activity:Measured by its ability to cleave a fluorogenic substrate, 4-Methylumbelliferyl alpha-L-iduronide.
Storage and Stability:May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 6 months after receipt at -20°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.
仕様
Size：10ug
Source Antigen：Recombinant, NSO cells
Grade：Highly Purified
Purity：≥95%, as determined by SDS-PAGE and visualized by silver stain. Endotoxin: ≤1EU/ug (LAL)
Form：Supplied as a liquid in 40mM sodium acetate, 400mM sodium chloride, pH 5.0, 20% glycerol.
