产品货号：
Z16903
中文名称：
GAA抗体
英文名称：
Anti-GAA Antibody
产品规格：
50μl|100μl|150μl
发货周期：
1～3天
产品价格：
询价
| 抗体名称 | Anti-GAA Antibody |
| 指标别名 | Acid maltase;Aglucosidase alfa;GAA;glucosidase;alpha;acid;LYAG;Lysosomal alpha glucosidase |
| 克隆性 | Polyclonal |
| 检验物种 | human |
| 应用范围 | WB,IHC |
| 基因名称 | GAA |
| 抗体来源 | Rabbit |
| 抗体类型 | IgG |
| 免疫原 | A synthetic peptide corresponding to a sequence in the middle region of human GAA (494-527aa TALAWWEDMVAEFHDQVPFDGMWIDMNEPSNFIR),different from the related mouse sequence by eight amino acids,and from the related rat sequence by six amino acids. |
| 实际分子量 | 110,95,76,70KD |
| 成分 | 500μg/mL antibody with PBS，0.02% NaN3,1mg BSA and 50% glycerol. |
| 纯化方式 | Immunogen affinity purified. |
| 浓度 | 500μg/mL |
| 产品形态 | 溶液 |
| 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 | Lysosomal alpha-glucosidase is an enzyme that in humans is encoded by the GAA gene.This gene encodes lysosomal alpha-glucosidase,which is essential for the degradation of glycogen to glucose in lysosomes.The encoded preproprotein is proteolytically processed to generate multiple intermediate forms and the mature form of the enzyme.Defects in this gene are the cause of glycogen storage disease II,also known as Pompe's disease,which is an autosomal recessive disorder with a broad clinical spectrum.Alternative splicing results in multiple transcript variants. |
| Uniprot ID | P10253 |
| 推荐配套试剂 | Biorab recommends Enhanced Chemiluminescent Kit with anti-Rabbit IgG (ZN1926) for Western blot,and HRP Conjugated anti-Rabbit IgG Super Vision Assay Kit (ZN1865) for IHC(P).*Blocking peptide可以联系我们购买。 |
| 推荐稀释比 | Western blot(WB) 1:500～2000 Immunohistochemistry in paraffin section (IHC) 1:50～400 (Boiling the paraffin sections in 10mM citrate buffer,pH6.0,or PH8.0 EDTA repair liquid for 20 mins is required for the staining of formalin/paraffin sections.) Optimal working dilutions must be determined by end user. |
相关搜索：GAA抗体，GAA多抗，Anti-GAA Antibody