{"product_id":"cst-49727t","title":"CST,  49727T, HEXB (E9X5S) Feline Chimeric Monoclonal Antibody","description":"Monoclonal Antibody for studying HEXB mouse. Validated for Immunofluorescence (Frozen). Available in 2 sizes. Highly specific and rigorously validated in-house, HEXB (E9X5S) Feline Chimeric Monoclonal Antibody (CST #49727) is ready to ship.\n\n\u003cb\u003eProduct Usage Information\u003c\/b\u003e\nImmunofluorescence (Frozen): 1:50 - 1:200\n\u003cb\u003eStorage\u003c\/b\u003e\nSupplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg\/mL BSA, 50% glycerol, and less than 0.02% sodium azide. Store at -20°C. Do not aliquot the antibody.\n\u003cb\u003eProtocol\u003c\/b\u003e\nAvailable protocols: Immunofluorescence (Frozen)\n\u003cb\u003eSpecificity \/ Sensitivity\u003c\/b\u003e\nHEXB (E9X5S) Feline Chimeric Monoclonal Antibody recognizes endogenous levels of total HEXB protein.\nSpecies Reactivity: Mouse, Rat\n\u003cb\u003eSource \/ Purification\u003c\/b\u003e\nThis recombinant chimeric antibody is engineered from HEXB (E9X5S) Rabbit mAb #33663 according to animal-free protocols. The chimeric antibody retains its antigen-binding Fab regions from the original rabbit monoclonal antibody but contains a feline-derived Fc domain. When multiplexing, Fc-directed rabbit secondaries are required to detect rabbit-host primary antibodies. The parent antibody, HEXB (E9X5S) Rabbit mAb #33663, 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\nSource\/Isotype: Feline chimeric IgG1","brand":"CST","offers":[{"title":"Default Title","offer_id":46800295526569,"sku":"49727T","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/products\/cst-49727t","provider":"Iright","version":"1.0","type":"link"}