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广州威佳科技有限公司
100ul
货号 | A00692-1 |
---|---|
产品名称 | Anti-HEXA Antibody |
基因名 | HEXA |
抗体来源 | Rabbit |
克隆 | Polyclonal |
抗体亚型 | Rabbit IgG |
分子量 | precursor61KD, mature54KD |
免疫原 | E.coli-derived human HEXA recombinant protein (Position: A135-H179). |
内容 | 500 ug/ml antibody with PBS ,0.02% NaN3 , 1 mg BSA and 50% glycerol. |
纯化方式 | Immunogen affinity purified. |
浓度 | 500 ug/ml |
产品形态 | Liquid |
保存条件 | 12 months from date of receipt,-20℃ as supplied. 6 months 2 to 8℃ after reconstitution. Avoid repeated freezing and thawing. |
背景资料 | HEXA, hexosaminidase A (alpha polypeptide), is an enzyme that in humans is encoded by the HEXA gene. Hexosaminidase A and the cofactor GM2 activator protein catalyze the degradation of the GM2 gangliosides and other molecules containing terminal N-acetyl hexosamines. The HEXA gene encodes the alpha subunit of hexosaminidase A , a lysosomal enzyme involved in the breakdown of gangliosides. The HEXA gene is mapped on 15q23. Even though the alpha and beta subunits of hexosaminidase A can both cleave GalNAc residues, only the alpha subunit is able to hydrolyze GM2 gangliosides. The alpha subunit contains a key residue, Arg-424, which is essential for binding the N-acetyl-neuramanic residue of GM2 gangliosides. Chimeric constructs were expressed in HeLa cells and selected constructs were produced in the baculovirus expression system to determine their ability to degrade GM2 ganglioside in the presence of GM2 activator protein. Their results allowed them to define 2 noncontiguous sequences in the alpha subunit (amino acids 1-191 and 403-529) which, when substituted into analogous positions in the beta subunit, conferred activity against the sulfated substrate. |
研究类别 | 1. Akli, S., Chomel, J.-C., Lacorte, J.-M., Bachner, L., Poenaru, A., Poenaru, L. Ten novel mutations in the HEXA gene in non-Jewish Tay-Sachs patients. Hum. Molec. Genet. 2: 61-67, 1993. 2. Beutler, E., Kuhl, W., Comings, D. Hexosaminidase isozyme in type O Gm2 gangliosidosis (Sandhoff-Jatzkewitz disease). Am. J. Hum. Genet. 27: 628-638, 1975.3. Chern, C. J., Beutler, E., Kuhl, W., Gilbert, F., Mellman, W. J., Croce, C. M. Characterization of heteropolymeric hexosaminidase A in human x mouse hybrid cells. Proc. Nat. Acad. Sci. 73: 3637-3640, 1976. |
Uniprot ID | HEXA: P06865 |
推荐配套的二抗和检测试剂 | Boster recommends Enhanced Chemiluminescent Kit with anti-Rabbit IgG (EK1002) for Western blot, and HRP Conjugated anti-Rabbit IgG Super Vision Assay Kit (SV0002-1) for ICC. |
为了提供优质的抗体,博士德对每一批抗体都用没有转染过的细胞系和体细胞组织检测,以保证博士德出品的抗体有足够的亲和性足以和对应蛋白天然的表达含量起反应。
应用 | 稀释度* |
---|---|
Western blot(WB): | 1:500-2000 |
Immunocytochemistry/Immunofluorescence (ICC/IF): | 1:50-400 |
(ELISA): | 1:100-1000 |
*稀释度需要用户自己调试,此处数据仅供参考。
**博士德提供高敏感的二抗和检测试剂盒。详情见相关产品推荐。
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