The Iduronidase, a-L- (alpha-L-Iduronidase, IDUA) Antibody from MyBioSource.com is a Human, and Mouse Monoclonal antibody to alpha-L-iduronidase, Esk, Esk1, IDA, and IDUA. This antibody recognizes Human, and Mouse antigen. The Iduronidase, a-L- (alpha-L-Iduronidase, IDUA) Antibody has been validated for the following applications: ELISA, and Western Blot.
Description
Alpha-L-Iduronidase (IDUA) encoded by the IDUA gene is an important enzyme required for the lysosomal degradation of glycosaminoglycans (GAGs). It hydrolyzes the nonreducing terminal alphaLiduronic 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