首页 > R&D Systems > R&D Systems/Human Hexosaminidase A/HEXA Antibody/MAB6237/100 ug

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R&D Systems/Human Hexosaminidase A/HEXA Antibody/MAB6237/100 ug

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MAB6237-100ug

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Species ReactivityHumanSpecificityDetects human Hexosaminidase A/HEXA in direct ELISAs and Western blots.SourceMonoclonal Mouse IgG2B Clone # 714729PurificationProtein A or G purified from hybridoma culture supernatantImmunogenS. frugiperda insect ovarian cell line Sf 21-derived recombinant human Hexosaminidase A/HEXAMet1-Thr529Accession # P06865FormulationLyophilized from a 0.2 μm filtered solution in PBS with Trehalose. *Small pack size (SP) is supplied as a 0.2 µm filtered solution in PBS.LabelUnconjugated Western Blot2 µg/mLSee belowImmunohistochemistry8-25 µg/mLSee belowReconstitutionSterile PBS to a final concentration of 0.5 mg/mL.ShippingThe product is shipped at ambient temperature. Upon receipt, store it immediately at the temperature recommended below. *Small pack size (SP) is shipped with polar packs. Upon receipt, store it immediately at -20 to -70 °CStability & StorageUse a manual defrost freezer and avoid repeated freeze-thaw cycles. 12 months from date of receipt, -20 to -70 °C as supplied.1 month, 2 to 8 °C under sterile conditions after reconstitution.6 months, -20 to -70 °C under sterile conditions after reconstitution. References:Gilbert, F. et al. (1975) Proc. Natl. Acad. Sci. USA 72:263. Myerowitz, R. et al. (1985) Proc. Natl. Acad. Sci. USA 82:7830. Korneluk, R.G. et al. (1986) J. Biol. Chem. 261:8407. Mark, B.L. et al. (2003) J. Mol. Biol. 327:1093. Mahuran, D.J. et al. (1988) J. Biol. Chem. 263:4612. Hepbildikler, S.T. et al. (2002) J. Biol. Chem. 277:2562. Mahuran, D.J. (1991) Biochim. Biophys. Acta 1096:87. Mencarelli, S. et al. (2005) FEBS Lett. 579:5501. Neufeld, E.F. (1989) J. Biol. Chem. 264:10927. Ohno, K. et al. (2008) Mol. Genet. Metab. 94:462. Entrez Gene IDs:3073 (Human)Alternate Names:beta-hexosaminidase subunit alpha; Beta-N-acetylhexosaminidase subunit alpha; EC 3.2.1; EC 3.2.1.52; HEXA; hexosaminidase A (alpha polypeptide); Hexosaminidase A; Hexosaminidase subunit A; MGC99608; N-acetyl-beta-glucosaminidase subunit alpha; TSDBackground:beta -hexosaminidases are enzymes involved in the hydrolysis of terminal N-acetyl-D-hexosamine residues in GM2 gangliosides and globo-sphingolipids in lysosomes (1‑4). The enzymes are composed of twoalphaand/orbetasubunits, which are coded by HEXA and HEXB genes, respectively. Different association of thealphaandbetasubunits gives rise tobeta ‑hexosaminidase isoforms A, B and S (Hex A, B and S) (5), which have the composition ofalphabeta,betabetaandalphaalpha, respectively. Our recombinant HEXA is presumably isoform Hex S, because onlyalphasubunit was expressed. Hex S is suggested to releases non‑reducing end N-acetylgalactosamine residues from dermatan sulfate, chondroitin sulfate and sulfated glycolipid SM2 (6). Recombinant HEXA is also highly active on 4-methylumbelliferyl-N-acetyl-beta -D-glucosaminide (6). Mutations in HEXA and HEXB genes cause lysosomal lipid storage disorders. Specifically, mutations of HEXA cause Tay-Sachs disease, manifested by the harmful accumulation of ganglioside GM2 in tissues and nerve cells in the brain (7‑10). Children with this disease usually die by age 4.

R&D Systems位于美国的明尼苏达州，一直致力于生物制品的开发与生产。公司成立之初主要生产用于医院及临床应用的血控品。1997年，公司推出第一个产品--富血小板血浆质控品；1981年，公司成为全球第二家生产含血小板全血控品的供应商。40多年的发展中，R&D Systems仍持续开发各种血控品产品。

1985年，作为公司推出的第一个科研试剂产品，也是全球第一家将该产品进行商业化生产的产品，R&D Systems成功上市了TGF-beta1蛋白。作为胞外信号分子，TGF-beta1蛋白在多种细胞中进行表达，并作为胞外信号分子参与免疫功能，细胞增殖和细胞分化等生物学过程。该产品推出后，公司陆续从生物材料中纯化了几种细胞因子产品并推向市场。

天然蛋白类产品的成功推广，加速了R&D Systems在细胞因子市场的开拓。公司于1985年形成Growth Factor事业部（现Biotechnology事业部）。Biotechnology事业部的目标是生产和营销重组人细胞因子。与天然来源提取蛋白相比较，DNA重组技术生产的蛋白产品其成本更低，产量更高，完全摆脱原料的限制。1989年，Biotechnology事业部开始开发抗细胞因子的单克隆和多克隆抗体，并于1990年开发了第一个ELISA试剂盒。

2014年2月10日，R&D Systems的母公司宣布命名为Bio-Techne. Bio-Techne旗下包括R&D Systems, Novus Biologicals, BiosPacific, Tocris Bioscience, Boston Biochem和Bionostics。