Human GLA ELISA Kit PicoKine®

This gene encodes a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. This enzyme predominantly hydrolyzes ceramide trihexoside, and it can catalyze the hydrolysis of melibiose into galactose and glucose. A variety of mutations in this gene affect the synthesis, processing, and stability of this enzyme, which causes Fabry disease, a rare lysosomal storage disorder that results from a failure to catabolize alpha-D-galactosyl glycolipid moieties.

Lots Lot 1 (pg/ml) Lot 2 (pg/ml) Lot 3 (pg/ml) Lot 4 (pg/ml) Mean (pg/ml) Standard Deviation CV (%) Sample 1 88 92 95 84 90 3.23 3.6% Sample 2 269 332 285 302 297 13.96 4.7% Sample 3 897 922 953 930 926 37.95 4.1% Lots Lot 1 (pg/ml) Lot 2 (pg/ml) Lot 3 (pg/ml) Lot 4 (pg/ml) Mean (pg/ml) Standard Deviation CV (%) Lots Lot 1 (pg/ml) Lot 2 (pg/ml) Lot 3 (pg/ml) Lot 4 (pg/ml) Mean (pg/ml) Standard Deviation CV (%) Sample 1 88 92 95 84 90 3.23 3.6% Sample 1 88 92 95 84 90 3.23 3.6% Sample 2 269 332 285 302 297 13.96 4.7% Sample 2 269 332 285 302 297 13.96 4.7% Sample 3 897 922 953 930 926 37.95 4.1% Sample 3 897 922 953 930 926 37.95 4.1%

Intra-Assay Precision Inter-Assay Precision Sample 1 2 3 1 2 3 n 16 16 16 24 24 24 Mean (pg/ml) 88 269 897 96 302 923 Standard deviation 5.37 17.49 39.47 4.51 16.31 54.46 CV (%) 6.1% 6.5% 4.4% 4.7% 5.4% 5.9% Intra-Assay Precision Inter-Assay Precision Sample 1 2 3 1 2 3 n 16 16 16 24 24 24 Mean (pg/ml) 88 269 897 96 302 923 Standard deviation 5.37 17.49 39.47 4.51 16.31 54.46 CV (%) 6.1% 6.5% 4.4% 4.7% 5.4% 5.9% Intra-Assay Precision Inter-Assay Precision Intra-Assay Precision Inter-Assay Precision Sample 1 2 3 1 2 3 Sample 1 2 3 1 2 3 n 16 16 16 24 24 24 n 16 16 16 24 24 24 Mean (pg/ml) 88 269 897 96 302 923 Mean (pg/ml) 88 269 897 96 302 923 Standard deviation 5.37 17.49 39.47 4.51 16.31 54.46 Standard deviation 5.37 17.49 39.47 4.51 16.31 54.46 CV (%) 6.1% 6.5% 4.4% 4.7% 5.4% 5.9% CV (%) 6.1% 6.5% 4.4% 4.7% 5.4% 5.9%

Description|Quantity Pre-coated 96-well strip microplate|1 Standard|2 vials Biotinylated antibody (100x) |100ul Avidin-Biotin-Peroxidase Complex (100x) |100ul Sample Diluent|30ml Antibody Diluent|12ml Avidin-Biotin-Peroxidase Diluent|12ml Color Developing Reagent (TMB) |10ml Stop Solution|10ml Wash Buffer (25x) |20ml Adhesive plate sealers|4

Store at 4°C for 6 months, at -20°C for 12 months. Avoid multiple freeze-thaw cycles (Ships with gel ice, can store for up to 3 days in room temperature. Freeze upon receiving.)

Concentration (pg/ml) 0 93.8 187.5 375 750 1500 3000 6000 O.D. 0.016 0.107 0.187 0.329 0.6 1.058 1.774 2.276 Concentration (pg/ml) 0 93.8 187.5 375 750 1500 3000 6000 O.D. 0.016 0.107 0.187 0.329 0.6 1.058 1.774 2.276 Concentration (pg/ml) 0 93.8 187.5 375 750 1500 3000 6000 Concentration (pg/ml) 0 93.8 187.5 375 750 1500 3000 6000 O.D. 0.016 0.107 0.187 0.329 0.6 1.058 1.774 2.276 O.D. 0.016 0.107 0.187 0.329 0.6 1.058 1.774 2.276

Microplate Reader capable of reading absorbance at 450nm. Incubator. Automated plate washer (optional) . Pipettes and pipette tips capable of precisely dispensing 0.5 μl through 1 ml volumes of aqueous solutions. Multichannel pipettes are recommended for large amount of samples. Deionized or distilled water. 500ml graduated cylinders. Test tubes for dilution.