Mucopolysaccharidosis type I (MPSI, Mc Kusick 252800) is an inherited autosomal recessive disease that results from a deficiency of α-L-iduronidase (IDUA; EC3.2.1.76) activity and results in progressive accumulation of dermatan and heparan sulfate, in lysosomes that may cause the clinical phenotype within a spectrum of severity ranging from severe Hurler syndrome to relatively mild Scheie syndrome (8-12). Here, IDUA is linked to mucopolysaccharidosis type 1.