Did you know: Hurler's Syndrome and Pseudo-Hurler's syndrome (1st April 2009)

Hurler's Syndrome:
It is an autosomal recessive catabolism disorder of the proteoglycans. Hydrolases that break the glycosaminoglycans present in the proteoglycans structures, inside the lysosomes are deficient. Due to this, products like dermatan sulphate and heparan sulphate accumulates in the lysosomes instead of being excreted in the urine.
Mental Retardation and mesenchymal defects are seen in the patient.

Pseudo-Hurler's syndrome:
It is due to the reduced availability of GlcNAc phosphtransferase that converts mannose to mannose-6-phosphate that is targeted to the lysosomes. Instead due to the fact that they are not converted they are sent out of the cell.
Psychomotor retardation and skeletal deformities are prominent.