Anti-HEXA (Beta-hexosaminidase Subunit alpha, Beta-N-acetylhexosaminidase Subunit alpha, Hexosaminid

B-hexosaminidases are enzymes involved in the hydrolysis of terminal N-acetyl-D-hexosamine residues in GM2 gangliosides and globo-sphingolipids in lysosomes. The enzymes are composed of two A and/or B subunits, which are coded by HEXA and HEXB genes, respectively. Different association of the A and B subunits gives rise to B-hexosaminidase isoforms A, B and S (Hex A, B and S), which have the composition of AB, BB and AA respectively. Our recombinant HEXA is presumably isoform Hex S, because only A subunit was expressed. Hex S is suggested to releases non-reducing end N-acetylgalactosamine residues from dermatan sulfate, chondroitin sulfate and sulfated glycolipid SM2. Recombinant HEXA is also highly active on 4-methylumbelliferyl-N-acetyl -B-D-glucosaminide. Mutations in HEXA and HEXB genes cause lysosomal lipid storage disorders. Specifically, mutations of HEXA cause Tay-Sachs disease, manifested by the harmful accumulation of ganglioside GM2 in tissues and nerve cells in the brain. Children with this disease usually die by age 4. Applications: Suitable for use in Western Blot and Immunohistochemistry. Other applications not tested. Recommended Dilution: Western Blot: 2ug/ml, Immunohistochemistry: 8-25ug/ml, Optimal dilutions to be determined by the researcher. Storage and Stability: Lyophilized powder may be stored at -20°C. Stable for 12 months at -20°C. Reconstitute with sterile PBS. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Reconstituted product is stable for 12 months at -20°C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.