Deficiency in α-L-Iduronidase activity leads to a condition known as [Mucopolysaccharidosis Type I (MPS I)](). MPS I is a lysosomal storage disorder characterized by the accumulation of undegraded GAGs in various tissues. The disorder presents in three clinical subtypes: Hurler syndrome, Hurler-Scheie syndrome, and Scheie syndrome, which vary in severity.
