| Product Name | Anti-HEXA (clone AT20F1) |
|---|---|
| Description | HEXA (Hexosaminidase A), also designated beta-Hexosaminidase A, is responsible for the degradation of GM2 gangliosides, and a variety of other molecules containing terminal N-acetyl hexosamines, in the brain and other tissues. A mutation in the a subunit of hexosaminidase is the cause of Tay-Sachs disease (TSD), also known as GM2-gangliosidosis type I. TSD is a fatal autosomal recessive lysosomal storage disease of the central nervous system (CNS) caused by insufficient activity of the HEXA enzyme that results in a failure to process GM2 gangliosides. The accumulation of GM2 ganglioside in the absence of HEXA activity causes progressive destruction of the CNS. |
| Synonyms | beta-hexosaminidase subunit alpha, TSD, hexosaminidase A |
| Host | Mouse |
| Clone | AT20F1. Anti-human HEXA mAb, clone AT20F1, is derived from hybridization of mouse F0 myeloma cells with spleen cells from BALB/c mice immunized with a recombinant human HEXA protein. |
| Immunogen | Recombinant human HEXA (89-529aa) purified from E. coli |
| Isotype | IgG2a,L |
| Reactivity | Human, Mouse |
| Applications | ELISA, WB, Dot Blot |
| Form | Purified, in Phosphate-Buffered Saline (pH 7.4) containing 10% Glycerol, 0.02% Sodium Azide. |
| Storage | Can be stored at +4C. For long term storage, aliquot and store at -20C. Avoid repeated freezing and thawing cycles. |
| References | Cabezas. J.A. (1989) Biochem J 261(3): 1059€“1060Lemieux. M.J., et al. (2006) J Mol Biol 359(4): 913€“929Boles. D.J. and Proia. R.L. (1995) Am J Hum Genet 56(3): 716€“724 |
| Supplier | ARP |
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