Human alpha -L-Iduronidase/IDUA Alexa Fluor® 647-conjugated Antibody
Human alpha -L-Iduronidase/IDUA Alexa Fluor® 647-conjugated Antibody Summary
Ala26-Pro653 (Ala26Thr)
Accession # P35475.2
Applications
Please Note: Optimal dilutions should be determined by each laboratory for each application. General Protocols are available in the Technical Information section on our website.
Preparation and Storage
Background: alpha-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). The sequence of human IDUA shares 79% amino acid identity with mouse IDUA.
Product Datasheets
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