ARYLSULFATASE B
Maroteaux Lamy Mucopolysaccharidosis, MPS VI
BIOCHEMICAL ANALYSIS

Description:

The deficiency of arylsulfatase B results in Maroteaux Lamy Mucopolysaccharidosis. This protocol utilizes a synthetic substrate, p-nitrocatechol sulfate. Following incubation, the quantity of p-nitrocatechol released is determined spectrophotometrically following alkalization of the sample. The calculations are complex due to the attempt to eliminate the contribution of other sulfatases to the activity.

Specimen Requirements:

The assay is performed on skin fibroblasts only. For fibroblasts,send two T25 flasks of confluent cells in an insulated container by overnight carrier.

Normal Range:

Skin Fibroblasts: 5.3-25.0 nmol/min/mg protein

Turnaround Time:

2-10 days
Skin fibroblasts assays are growth dependent.

CPT Codes and Prices:

Skin Fibroblast Culture: 82657, 84311, 84155, 88240, 88233

Shipping Information

Forms:

>> Biochemical Requisition

Test Codes:

4540