{"product_id":"cst-28112sf","title":"CST,  28112SF, HEXB (E9X5S) Rabbit Monoclonal Antibody (BSA and Azide Free)","description":"Monoclonal Antibody for studying HEXB mouse. Validated for WB,IHC,IF,IF. Highly specific and rigorously validated in-house, HEXB (E9X5S) Rabbit Monoclonal Antibody (BSA and Azide Free) (CST #28112) is ready to ship.\n\n\u003cb\u003eProduct Usage Information\u003c\/b\u003e\nThis product is the carrier free version of product #33663. All data were generated using the same antibody clone in the standard formulation which contains BSA and glycerol. This formulation is ideal for use with technologies requiring specialized or custom antibody labeling, including fluorophores, metals, lanthanides, and oligonucleotides. It is not recommended for ChIP, ChIP-seq, CUT\u0026amp;RUN or CUT\u0026amp;Tag assays. If you require a carrier free formulation for chromatin profiling, please contact us . Optimal dilutions\/concentrations should be determined by the end user. BSA and Azide Free antibodies are quality control tested by size exclusion chromatography (SEC) to determine antibody integrity.\n\u003cb\u003eFormulation\u003c\/b\u003e\nSupplied in 1X PBS (10 mM Na 2 HPO 4 , 3 mM KCl, 2 mM KH 2 PO 4 , and 140 mM NaCl (pH 7.8)). BSA and Azide Free. For standard formulation of this product see product # 33663\n\u003cb\u003eStorage\u003c\/b\u003e\nStore at -20°C. This product will freeze at -20°C so it is recommended to aliquot into single-use vials to avoid multiple freeze\/thaw cycles. A slight precipitate may be present and can be dissolved by gently vortexing. This will not interfere with antibody performance.\n\u003cb\u003eSpecificity \/ Sensitivity\u003c\/b\u003e\nHEXB (E9X5S) Rabbit Monoclonal Antibody (BSA and Azide Free) recognizes endogenous levels of total HEXB protein.\nSpecies Reactivity: Mouse, Rat\n\u003cb\u003eSource \/ Purification\u003c\/b\u003e\nMonoclonal antibody is produced by immunizing animals with a synthetic peptide corresponding to residues surrounding Pro300 of mouse HEXB protein.\n\u003cb\u003eBackground\u003c\/b\u003e\nβ-hexosaminidase (Hex) is an important lysosomal enzyme system that degrades various cellular substrates, such as oligosaccharides, glycosaminoglycans, and glycolipids. It is encoded by two genes, and , respectively, and these subunits dimerize to form β-hexosaminidase A (HexA; αβ) and β-hexosaminidase B (HexB; ββ) (1). Loss-of-function mutations result in ganglioside (GM2) accumulation and progressive neurodegenerative diseases, such as Sandhoff disease (SD) and Tay-Sachs disease (TSD) (1). HexB knockout mice display, similarly to human patients, a near-normal phenotype at birth but quickly develop muscle weakness, rigidity, and motor deterioration, typically leading to death at approximately four months of age (2). It has been shown that loss of HEXB leads to reduction of early neuronal migration and differentiation in the embryonic cerebral cortices of these mice (3). Hex also plays an important role in cancer, being a new potential biomarker in laryngeal cancer. Furthermore, higher expression of HEXA and HEXB is associated with poor prognosis in glioblastoma multiforme (GBM) patients (1).\n\u003cb\u003eAlternate Names\u003c\/b\u003e\nBeta-hexosaminidase subunit beta; Beta-N-acetylhexosaminidase subunit beta; Hexb; hexosaminidase B; Hexosaminidase subunit B; N-acetyl-beta-glucosaminidase subunit beta\n\n\u003cb\u003eSpecification\u003c\/b\u003e\n\nREACTIVITY: M R\nSENSITIVITY: Endogenous\nMW (kDa): 52, 70\nSource\/Isotype: Rabbit IgG","brand":"CST","offers":[{"title":"Default Title","offer_id":46800086597801,"sku":"28112SF","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/products\/cst-28112sf","provider":"Iright","version":"1.0","type":"link"}