HEXA

Size

£652.00

SKU:: 030296
Purity:: Purified
Storage Conditions:: -20°C
Supplier:: United States Biological
Host:: Mouse
Immunogen:: Purified recombinant fragment of human HEXA expressed in E. coli.
Clone:: 13B374
Format:: Purified antibody in PBS with 0.05% sodium azide and 0.5% protein stabilizer.
Extra Details:: This gene encodes the alpha subunit of the lysosomal enzyme beta-hexosaminidase that, together with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules containing terminal N-acetyl hexosamines. Beta-hexosaminidase is composed of two subunits, alpha and beta, which are encoded by separate genes. Both beta-hexosaminidase alpha and beta subunits are members of family 20 of glycosyl hydrolases. Mutations in the alpha or beta subunit genes lead to an accumulation of GM2 ganglioside in neurons and neurodegenerative disorders termed the GM2 gangliosidoses. Alpha subunit gene mutations lead to Tay-Sachs disease (GM2-gangliosidosis type I). Applications: Western Bloting: 1:500-1:2000. Flow cytometry: 1:200-1:400. ELISA: Suggested dilution 1:10000. Optimal dilutions to be determined by the researcher. Storage and Stability: May be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20°C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.