Human Hexosaminidase A/HEXA MAb (Clone 714712) (25 UG)【科瑞奥博】

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Human Hexosaminidase A/HEXA MAb (Clone 714712) (25 UG)

货号： MAB62371-SP 基本售价： 1467.3 元规格： 25 ug

产品信息

概述
货号	MAB62371-SP
别名	beta-hexosaminidase subunit alpha; Beta-N-acetylhexosaminidase subunit alpha; EC 3.2.1; EC 3.2.1.52; HEXA; hexosaminidase A (alpha polypeptide); Hexosaminidase subunit A; MGC99608; N-acetyl-beta-glucosaminidase subunit alpha; TSD
反应种属	Human
应用	Neutralization
目标/特异性	Detects human Hexosaminidase A/HEXA in direct ELISAs.
使用方法	Neutralization: Measured by its ability to neutralize Human Hexosaminidase A (2.0 μg/mL,Catalog # 6237-GH) cleavage of the fluorogenic peptide substrate 4-Methylumbelliferyl-N-Acetyl-beta -D-glucosaminide (400 µM). The Neutralization Dose (ND₅₀) is typically 3.0 µg/mL.
来源	Monoclonal Mouse IgG_2B Clone # 714712
产品组分

性能
供应商	R&D Systems
Entrez Gene IDs	3073 (Human)
纯化方式	Protein A or G purified from hybridoma culture supernatant
免疫原	S. frugiperda insect ovarian cell line Sf21-derived recombinant human Hexosaminidase A/HEXA Met1-Thr529 Accession # P06865
生物活性	Human
标记	Unconjugated
溶解方法	Sterile PBS to a final concentration of 0.5 mg/mL.
背景	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 two alpha and/or beta subunits, which are coded by HEXA and HEXB genes, respectively. Different association of the alpha and beta subunits gives rise to beta ‑hexosaminidase isoforms A, B and S (Hex A, B and S) (5), which have the composition of alpha beta, beta beta and alpha alpha, respectively. 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.
运输条件	Blue Ice
存放说明	4℃
参考文献	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.

参考图片

Neutralization of Human Hexosaminidase A Activity by Human Hexosaminidase A Antibody. The cleavage of 4-Methylumbelliferyl-N-Acetyl-beta -D-glucosaminide (400 μM) by Human Hexosaminidase A (2.0 μg/mL,Catalog # 6237-GH) is measured after it has been preincubated with increasing concentrations of Mouse Anti-Human Hexosaminidase A Monoclonal Antibody (Catalog # MAB62371). The ND50 is typically 3.0 μg/mL.