The enzyme participates in the degradation of dermatan sulfate. In the deficiency state, mucopolysaccharides accumulate in lysosomes eventually results in disruption of the integrity of the cell. Our protocol utilizes a synthetic substrate, p -nitrocatechol sulfate. Following incubation, the quantity of p -nitrocatechol released is determined spectrophotometrically following alkalization of the sample. Turn around time is 3-5 days.
Prenatal diagnosis is available on chorionic villus cells or amniocytes.
Prior contact with the laboratory is required before submitting any samples for prenatal diagnosis. Turn around time is dependent on cell growth but is usually 1-2 weeks. You may return to search anew or return to UCSD Biochemical Genetics home page.