A person cannot break down long chains of sugar molecules (GAG's)

An abnormal accumulation of extracellular proteoglycans and secretion of dermatan sulfate and heparan sulfate into the urine.

Hurler's Syndrome leads to bone and cartilage abnormalities and mental retardation. Leads to abnormal bones in the spine, claw hand, cloudy corneas, deafness, halted growth, and joint disease, including stiffness.

Leads to heart valve problems (GAG's buildup and damage organs).

Mucopolysaccharidosis Type I

- In Hurler's Syndrome, failure to metabolize certain glycosaminoglycans causes the accumulation of large amounts of matrix within connective tissue, which distorts growth of many parts of the body.