Patients with Hurler syndrome (complete deficiency of α-L-iduronidase) have mental deficiency in addition to physical deformities, corneal clouding, and valvular heart disease.Their mental deficiency is caused by the accumulation of:

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Heparan sulfate is the only GAG that is common in the brain.Its accumulation is responsible for mental deficiency in many mucopolysaccharidoses.

Patients with Hurler Syndrome (Complete Deficiency of α-L-Iduronidase) Have Mental