Human IDUA ELISA Kit

Supplementary info

This supplementary information is collated from multiple sources and compiled automatically.

Activity summary

IDUA also known as alpha-L-iduronidase functions as an enzyme responsible for the breakdown of glycosaminoglycans. Its mechanical role involves the hydrolysis of the terminal α-L-iduronic acid residues essential in the degradation process of heparan sulfate and dermatan sulfate. The average molecular mass of IDUA is approximately 72 kDa. It expresses predominantly in lysosomes the cell's compartments that deal with digestion and waste processing. IDUA performs its functions most notably in tissues with high turnover of glycosaminoglycans such as liver spine and connective tissue.

Biological function summary

Alpha-L-iduronidase plays an important role in maintaining normal cellular operations by catalyzing important steps of the lysosomal degradation pathway. It is not part of a complex but functions independently within the lysosome. Through its enzymatic activity IDUA prevents the accumulation of undegraded molecules which could otherwise disrupt cellular function. The proper function of IDUA maintains cellular homeostasis illustrating its necessity for regular cellular maintenance.

Pathways

IDUA integrates most prominently into the lysosomal storage pathway due to its fundamental role in glycosaminoglycan degradation. This pathway includes other related enzymes like iduronate-2-sulfatase which work synergistically to ensure the complete breakdown of glycosaminoglycans. Another notable pathway where IDUA plays a part is the catabolism of carbohydrates linking it to various carbohydrate-active enzymes that collaboratively process complex sugar molecules.

Associated diseases and disorders

Mutations or deficiencies in IDUA can result in significant health conditions such as Mucopolysaccharidosis type I (MPS I) which includes Hurler Hurler-Scheie and Scheie syndromes. These disorders arise from the accumulation of heparan sulfate and dermatan sulfate leading to a wide range of symptoms affecting multiple organs. IDUA's dysfunction connects to another enzyme iduronate-2-sulfatase which when also deficient could further exacerbate the accumulation of glycosaminoglycans and worsen clinical outcomes.