{"product_id":"cst-33663t","title":"CST,  33663T, HEXB (E9X5S) Rabbit Monoclonal Antibody","description":"Monoclonal Antibody for studying HEXB mouse. Validated for WB,IHC,IF,IF. Available in 2 sizes. Highly specific and rigorously validated in-house, HEXB (E9X5S) Rabbit Monoclonal Antibody (CST #33663) is ready to ship.\n\n\u003cb\u003eProduct Usage Information\u003c\/b\u003e\nWestern Blotting: 1:1000\nImmunohistochemistry (Paraffin): 1:100 - 1:400\nImmunofluorescence (Frozen): 1:800 - 1:3200\nImmunofluorescence (Immunocytochemistry): 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. For a carrier free (BSA and azide free) version of this product see product # 28112 .\n\u003cb\u003eProtocol\u003c\/b\u003e\nAvailable protocols: Western Blotting, Immunohistochemistry (Paraffin), Immunofluorescence (Frozen), Immunofluorescence (Immunocytochemistry)\n\u003cb\u003eSpecificity \/ Sensitivity\u003c\/b\u003e\nHEXB (E9X5S) Rabbit Monoclonal Antibody recognizes endogenous levels of total HEXB protein. This antibody is unable to detect HEXB in some low-expressing cell lines by immunofluorescence.\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":46800089776297,"sku":"33663T","price":0.99,"currency_code":"USD","in_stock":true}],"url":"https:\/\/iright.com\/products\/cst-33663t","provider":"Iright","version":"1.0","type":"link"}