b-GLUCOSIDASE
Gaucher Disease
BIOCHEMICAL ANALYSIS
Also see: Gaucher Disease - DNA Analysis

Description:

The deficiency of b-D-Glucosidase results in Gaucher disease. This assay utilizes the synthetic substrate, 4-methylumbelliferyl-b-D-glucoside, which is hydrolyzed to release 4-methylumbelliferone. Activity is determined by measuring the quantity of 4-methylumbelliferone formed using the fluorimeter. For Ashkenazi Jewish individuals, DNA analysis is also available (see Gaucher disease).

Specimen Requirements:

The assay is performed on white blood cells or cultured skin fibroblasts. For white blood cells, draw 7-10 ml in a yellow top (ACD) tube and ship to us on wet ice in an insulated container by overnight express. Sample must arrive at our location within 48 hrs. For fibroblasts, send two T25 flasks of confluent cells in an insulated container by overnight carrier.

Normal Range:

Skin Fibroblasts: 3.0-10.0 nmol/min/mg protein
White Blood Cells: 98-208 pmol/min/mg protein

Turnaround Time:

2-10 days
Skin fibroblasts assays are growth dependent.

CPT Codes and Prices:

Skin Fibroblast Culture: 82657, 84155, 88240, 88233
White Blood Cells: 82657, 84155

Shipping Information

Forms:

>> Biochemical Requisition

Test Codes:

Skin Fibroblast Culture: 4553
White Blood Cells: 4554