Human HEXA ELISA Kit PicoKine®

Hexosaminidase A (alpha polypeptide), also known as HEXA, is an enzyme that in humans is encoded by the HEXA gene, located on the 15th chromosome. This gene encodes a member of the glycosyl hydrolase 20 family of proteins. The encoded preproprotein is proteolytically processed to generate the alpha subunit of the lysosomal enzyme beta-hexosaminidase. This enzyme, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Mutations in this gene lead to an accumulation of GM2 ganglioside in neurons, the underlying cause of neurodegenerative disorders termed the GM2 gangliosidoses, including Tay-Sachs disease (GM2-gangliosidosis type I) . Alternative splicing results in multiple transcript variants, at least one of which encodes a preproprotein that is proteolytically processed.

Lots Lot 1 (pg/ml) Lot 2 (pg/ml) Lot 3 (pg/ml) Lot 4 (pg/ml) Mean (pg/ml) Standard Deviation CV (%) Sample 1 528 498 562 525 528 31.17 5.9% Sample 2 1757 1716 1694 1721 1722 91.27 5.3% Sample 3 4955 4633 4851 4947 4847 247.17 5.1% 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 1 528 498 562 525 528 31.17 5.9% Sample 1 528 498 562 525 528 31.17 5.9% Sample 2 1757 1716 1694 1721 1722 91.27 5.3% Sample 2 1757 1716 1694 1721 1722 91.27 5.3% Sample 3 4955 4633 4851 4947 4847 247.17 5.1% Sample 3 4955 4633 4851 4947 4847 247.17 5.1%

Intra-Assay Precision Inter-Assay Precision Sample 1 2 3 1 2 3 n 16 16 16 24 24 24 Mean (pg/ml) 528 1757 4955 552 1694 4751 Standard deviation 20.59 89.61 237.84 28.70 76.23 242.3 CV (%) 3.9% 5.1% 4.8% 5.2% 4.5% 5.1% Intra-Assay Precision Inter-Assay Precision Sample 1 2 3 1 2 3 n 16 16 16 24 24 24 Mean (pg/ml) 528 1757 4955 552 1694 4751 Standard deviation 20.59 89.61 237.84 28.70 76.23 242.3 CV (%) 3.9% 5.1% 4.8% 5.2% 4.5% 5.1% 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) 528 1757 4955 552 1694 4751 Mean (pg/ml) 528 1757 4955 552 1694 4751 Standard deviation 20.59 89.61 237.84 28.70 76.23 242.3 Standard deviation 20.59 89.61 237.84 28.70 76.23 242.3 CV (%) 3.9% 5.1% 4.8% 5.2% 4.5% 5.1% CV (%) 3.9% 5.1% 4.8% 5.2% 4.5% 5.1%

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 (ng/ml) 0 0.312 0.62 1.25 2.5 5 10 20 O.D. 0.039 0.084 0.128 0.225 0.414 0.769 1.345 2.16 Concentration (ng/ml) 0 0.312 0.62 1.25 2.5 5 10 20 O.D. 0.039 0.084 0.128 0.225 0.414 0.769 1.345 2.16 Concentration (ng/ml) 0 0.312 0.62 1.25 2.5 5 10 20 Concentration (ng/ml) 0 0.312 0.62 1.25 2.5 5 10 20 O.D. 0.039 0.084 0.128 0.225 0.414 0.769 1.345 2.16 O.D. 0.039 0.084 0.128 0.225 0.414 0.769 1.345 2.16

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.