Table 1.

Composition of the lysosomal enzyme assay mixtures.

Assay	Assay volume, μL	Substrate, mmol/L	Internal standard, nmol	Buffer	Detergent	Additional components
ABG	25	0.2	0.2	0.37 mol/L citrate-phosphate, pH 5.1	9.6 g/L sodium taurocholate	None
ASM	25	0.2	0.1	0.55 mol/L sodium acetate, pH 5.5	0.6 g/L sodium taurocholate	None
GALC	25	0.2	0.1	0.18 mol/L citrate-phosphate, pH 4.5	9.6 g/L sodium taurocholate	0.96 g/L oleate
GAA	25	0.4	0.1	0.18 mol/L citrate-phosphate, pH 4.0	0.6 g/L Triton X-100	80 μmol/L acarbose
GLA	12.5	2.0	0.1	0.34 mol/L sodium acetate, pH 4.6	1.2 g/L sodium taurocholate	70 mmol/L N-acetylgalactosamine