Recombinant protein corresponding to Ala26-Pro653 of human alpha-L-Iduronidase expressed in NS0 cells.
Alpha-L-Iduronidase (IDUA) 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). MPS I causes progressive cellular, tissue and organ damage, and several clinical studies using enzyme replacement therapy have shown promising benefits. The sequence of human IDUA shares 79% aa identity with mouse IDUA. Applications: Suitable for use in Direct ELISA and Western Blot. Other applications not tested. Recommended Dilutions: Western Blot: 2ug/ml using human kidney tissue and IMR-90 human kung fibroblast cell line Optimal dilutions to be determined by the researcher. Storage and Stability: Lyophilized and reconstituted products are stable for 12 months after receipt at -20C. Reconstitute with sterile PBS. Aliquot to avoid repeated freezing and thawing. Store at -20C. 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.
