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Antigenic Specificity | Hexosaminidase A/HEXA, Human |
Clone | AT20F1 |
Host Species | Mouse |
Reactive Species | human |
Isotype | IgG2a Lambda |
Format | Protein A purified |
Size | 50ul, 100ul |
Concentration | 1mg/ml (determined by BCA assay) |
Applications | ELISA,WB,FACS |
Reviews / Ratings | If you have used this antibody, please help fellow researchers by submitting reviews to pAbmAbs and antYbuddY. |
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. |
Immunogen | Recombinant human HEXA (89-529aa) purified from E. coli |
Other Names | beta-hexosaminidase subunit alpha, TSD, hexosaminidase A |
Gene, Accession # | NCBI: NP_000511.1, UniProt: P06865 |
Catalog # | ATGA0259 |
Price | $170, $290 |
Order / More Info | Hexosaminidase A/HEXA, Human Antibody from NKMAX Co. Ltd. |
Product Specific References | n/a |