HEXB Rabbit pAb
Cat No.: APA3257
Size：
| Product Name: | HEXB Rabbit pAb |
| Cat No.: | APA3257 |
| source: | Rabbit |
| reactivity: | Human |
| applications: | WB |
| clonality: | Polyclonal |
| recommended dilution: | WB: 1:1000-1:5000 |
| format: | Liquid |
| isotype: | IgG |
| immunogen: | A synthetic peptide of human HEXB |
| calculated molecular weight: | 63 kDa |
| observed molecular weight: | 63 kDa |
| genbank accession number: | P07686 |
| gene id (ncbi): | 3074 |
| purification method: | Affinity Purification |
| conjugate: | Un-conjugated |
| storage: | Store at -20°C. Supplied in 50nM Tris-Glycine(pH 7.4), 0.15M NaCl, 40%Glycerol, 0.01% sodium azide and 0.05% BSA. Stable for 12 months from date of receipt. |
| synonyms: | ENC-1AS; HEL-248; HEL-S-111 |
| category: | Primary Ab |
| concentration: | 0.5mg/ml |
| background: | Hexosaminidase B is the beta 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. Beta subunit gene mutations lead to Sandhoff disease (GM2-gangliosidosis type II). Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, May 2014] |