Iduronidase

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Iduronidase

Iduronidase (pronounced as ih-doo-ROH-ni-dase) is an enzyme that plays a crucial role in the breakdown of certain complex molecules in the body. It is specifically involved in the degradation of a group of sugars called glycosaminoglycans (GAGs).

Etymology

The term "Iduronidase" is derived from the name of the sugar molecule it acts upon, "iduronic acid", and the suffix "-ase", which is used in biochemistry to denote an enzyme.

Function

Iduronidase is a lysosomal enzyme, meaning it is found in the lysosome, a specialized compartment within cells that is responsible for breaking down waste materials and cellular debris. It catalyzes the hydrolysis of the terminal iduronic acid residues of dermatan sulfate and heparan sulfate, two types of GAGs.

Clinical Significance

Deficiency of iduronidase leads to a rare genetic disorder known as Mucopolysaccharidosis type I (MPS I). This condition is characterized by the accumulation of GAGs in various tissues and organs, leading to a wide range of health problems, including skeletal abnormalities, heart valve disease, and developmental delay.

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