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Gene : |
HEXA |
Gene ID : |
3073 |
Uniprot ID : |
P06865 |
Alternative Name : |
TSD, hexosaminidase A, Beta-hexosaminidase subunit alpha, Beta-N-acetylhexosaminidase subunit alpha, Hexosaminidase subunit A, N-acetyl-beta-glucosaminidase subunit alpha. |
Format : |
Purified |
Amount : |
20 µg |
Clone name : |
PAT20F1Ae |
Isotype : |
Mouse IgG2a heavy chain and Lambda light chain. |
Immunogen Information : |
Anti-human HEXA mAb, clone PAT20F1A, is derived from hybridization of mouse F0 myeloma cells with spleen cells from BALB/c mice immunized with a recombinant human HEXA protein 89-529 amino acids purified from E. coli. |
HEXA is the alpha subunit of the lysosomal enzyme beta-hexosaminidase which, combined with the cofactor GM2 activator protein, catalyzes the degradation of the ganglioside GM2, and other molecules having N-acetyl hexosamines terminus. The two subunits composing Beta-hexosaminidase, alpha and beta, belong to the glycosyl hydrolases family and are encoded by distinct genes. Alpha subunit gene mutations can cause Tay-Sachs disease (GM2-gangliosidosis type I).
The antibody has been tested by ELISA, Western blot analysis to assure specificity and reactivity. Since application varies, however, each investigation should be titrated by the reagent to obtain optimal results. Recommended starting dilution is 1:3000.