alpha-L-iduronidase
alpha-L-iduronidase is an enzyme that plays a crucial role in breaking down specific complex carbohydrates known as glycosaminoglycans. It is primarily involved in the degradation of heparan sulfate and dermatan sulfate, which are important for various biological functions in the body.
Deficiency of alpha-L-iduronidase leads to a genetic disorder called Hurler syndrome, a type of mucopolysaccharidosis. This condition results in the accumulation of glycosaminoglycans in the body, causing various health issues, including developmental delays and organ dysfunction. Enzyme replacement therapy is one treatment option for managing this disorder.