| Applications: | ELISA, WB, FACS |
| Species Reactivity: | Human |
| Isotype: | Mouse IgG2a lamda |
| Immunogen: | Recombinant human HEXA (89-529aa) purified from E. coli |
| Purification: | By protein-A affinity chromatography |
| Conjugation: | Unconjugated |
| 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. |
| Further Information: | 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. |
| Synonym: | beta-hexosaminidase subunit alpha, TSD, hexosaminidase A |
| Ascension Number: | P06865 |
| NCBI EntrezGene: | NP_000511.1 |
| Shipping Conditions: | Ship on cold packs |
| Storage: | Can be stored at +2C to +8C for 1 week. For long term storage, aliquot and store at -20C to -80C. Avoid repeated freezing and thawing cycles. |
| Usage: | Research use only |
