Hurler syndrome is due to deficiency of
Heparitin Sulphate Isolated from amyloid liver, ceain normal tissues such as human and cattle aoa, and from the urine, liver, and spleen of patients with gargoylism (Hurler's syndrome). This compound has negligible anticoagulant activity. It seems to be structurally similar to heparin, but has a:Lower molecular weight,Some of the amino groups carry acetyl groups and percentage of -SO4 groups are smaller.Unlike heparin, its predominant uronic acid is D-Glucuronic acid (Glc UA).Recently it has been shown that it is present on cell surfaces as proteoglycan and is extracellular.Ref: M.N. Chatterjea - Textbook of Biochemistry, 8th edition, page no: 40
Beta galactosidase
Sphingomyelinase
Heparitin Sulphate
Hyaluronidase
Metabolism of lipid