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- BOSTER
- A01548-50ul
- 中国
- 2025年07月11日
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- 库存：
大量
- 供应商：
广州威佳科技有限公司
- 规格：
50ul
产品概况
| 货号 | A01548 |
|---|---|
| 产品名称 | Anti-GAA Antibody |
| 基因名 | GAA |
| 抗体来源 | Rabbit |
| 克隆 | Polyclonal |
| 抗体亚型 | Rabbit IgG |
| 分子量 | 110,95,76,70KD |
| 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. |
| 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 | 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. |
| 研究类别 | 1. "Entrez Gene: GAA glucosidase, alpha; acid (Pompe disease, glycogen storage disease type II)".2. Donald J. Voet; Judith G. Voet; Charlotte W. Pratt (2008). "Additional Pathways in Carbohydrate Metabolism". Principles of Biochemistry, Third edition. Wiley. p. 538.3. Reuser AJ, Kroos MA, Hermans MM, et al. (1995). "Glycogenosis type II (acid maltase deficiency).". Muscle Nerve. 3: S61–9. |
| Uniprot ID | GAA: P10253 |
| 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 IHC(P). *Blocking peptide 可以联系我们购买。 |
产品应用细节
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 |
| 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. | |
*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。
产品图片描述
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[list_product_images]Figure 1. Western blot analysis of GAA using anti- GAA antibody (A01548).
Lane 1: human A549 whole cell lysates,
Lane 2: human HepG2 whole cell lysates,
Lane 3: human HEK293 whole cell lysates,
Lane 4: human PC-3 whole cell lysates,
probed with a goat anti-rabbit IgG-HRP secondary antibody The signal is developed using an Enhanced Chemiluminescent detection (ECL) kit (Catalog # EK1002) . A specific band was detected for GAA at approximately 110,95,76,70KD. The expected band size for GAA is at 110,95,76,70KD.|Figure 2. IHC analysis of GAA using anti-GAA antibody (A01548).GAA was detected in paraffin-embedded section of human liver cancer tissues. .Biotinylated goat anti-rabbit IgG was used as secondary antibody . The tissue section was developed using Strepavidin-Biotin-Complex (SABC)(Catalog # SA1022) with DAB as the chromogen. |Figure 3. IHC analysis of GAA using anti-GAA antibody (A01548).GAA was detected in paraffin-embedded section of human prostatic cancer tissues. Biotinylated goat anti-rabbit IgG was used as secondary antibody . The tissue section was developed using Strepavidin-Biotin-Complex (SABC)(Catalog # SA1022) with DAB as the chromogen. [/list_product_images]
Lane 1: human A549 whole cell lysates,
Lane 2: human HepG2 whole cell lysates,
Lane 3: human HEK293 whole cell lysates,
Lane 4: human PC-3 whole cell lysates,
probed with a goat anti-rabbit IgG-HRP secondary antibody The signal is developed using an Enhanced Chemiluminescent detection (ECL) kit (Catalog # EK1002) . A specific band was detected for GAA at approximately 110,95,76,70KD. The expected band size for GAA is at 110,95,76,70KD.|Figure 2. IHC analysis of GAA using anti-GAA antibody (A01548).GAA was detected in paraffin-embedded section of human liver cancer tissues. .Biotinylated goat anti-rabbit IgG was used as secondary antibody . The tissue section was developed using Strepavidin-Biotin-Complex (SABC)(Catalog # SA1022) with DAB as the chromogen. |Figure 3. IHC analysis of GAA using anti-GAA antibody (A01548).GAA was detected in paraffin-embedded section of human prostatic cancer tissues. Biotinylated goat anti-rabbit IgG was used as secondary antibody . The tissue section was developed using Strepavidin-Biotin-Complex (SABC)(Catalog # SA1022) with DAB as the chromogen. [/list_product_images]
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Anti-GAA Antibody(A01548-50ul)
￥1180
