| 產(chǎn)品編號(hào) | bs-13254R |
| 英文名稱 | GAA Rabbit pAb |
| 中文名稱 | α葡萄糖苷酶/溶酶體α-葡糖苷酶抗體 |
| 別 名 | 70 kDa lysosomal alpha-glucosidase; Acid alpha glucosidase; Acid maltase; Aglucosidase alfa; alpha glucosidase; GAA; Glucosidase alpha acid(Pompe disease glycogen storage disease type II); Glucosidase alpha acid; Glucosidase alpha; LYAG; LYAG_HUMAN; Lysos |
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Specific References (1) | bs-13254R has been referenced in 1 publications.
[IF=5.23] Rauniyar et al. Quantitative Proteomics of Human Fibroblasts with I1061T Mutation in Niemann-Pick C1 (NPC1) Protein Provides Insights into the Disease Pathogenesis. (2015) Mol.Cell.Proteomics. 14:1734-49 WB ; Human.
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| 研究領(lǐng)域 | 腫瘤 細(xì)胞生物 信號(hào)轉(zhuǎn)導(dǎo) 新陳代謝 表觀遺傳學(xué) |
| 抗體來源 | Rabbit |
| 克隆類型 | Polyclonal |
| 交叉反應(yīng) | Human,Mouse (predicted: Rat,Pig,Sheep,Dog,Horse) |
| 產(chǎn)品應(yīng)用 | IHC-P=1:100-500,IHC-F=1:100-500,IF=1:200-800
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理論分子量 | 83/92/98 kDa |
| 檢測(cè)分子量 | |
| 細(xì)胞定位 | 細(xì)胞漿 細(xì)胞膜 |
| 性 狀 | Liquid |
| 濃 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human GAA/Glucosidase alpha: 541-640/952 |
| 亞 型 | |
| 純化方法 | affinity purified by Protein A |
| 緩 沖 液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 保存條件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事項(xiàng) | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| PubMed | PubMed |
| 產(chǎn)品介紹 |
This gene encodes acid alpha-glucosidase, which is essential for the degradation of glycogen to glucose in lysosomes. Different forms of acid alpha-glucosidase are obtained by proteolytic processing. 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. Three transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Jul 2008]. Function: Essential for the degradation of glygogen to glucose in lysosomes. Subcellular Location: Lysosome. Lysosome membrane. Post-translational modifications: The different forms of acid glucosidase are obtained by proteolytic processing. Phosphorylation of mannose residues ensures efficient transport of the enzyme to the lysosomes via the mannose 6-phosphate receptor. DISEASE: Defects in GAA are the cause of glycogen storage disease type 2 (GSD2) [MIM:232300]; also called acid alpha-glucosidase (GAA) deficiency or acid maltase deficiency (AMD). GSD2 is a metabolic disorder with a broad clinical spectrum. The severe infantile form, or Pompe disease, presents at birth with massive accumulation of glycogen in muscle, heart and liver. Cardiomyopathy and muscular hypotonia are the cardinal features of this form whose life expectancy is less than two years. The juvenile and adult forms present as limb-girdle muscular dystrophy beginning in the lower limbs. Final outcome depends on respiratory muscle failure. Patients with the adult form can be free of clinical symptoms for most of their life but finally develop a slowly progressive myopathy. Similarity: Belongs to the glycosyl hydrolase 31 family. Contains 1 P-type (trefoil) domain. SWISS: P10253 Gene ID: 2548 Database links: Entrez Gene: 2548 Human Entrez Gene: 14387 Mouse Omim: 606800 Human SwissProt: P10253 Human SwissProt: P70699 Mouse Unigene: 1437 Human Unigene: 4793 Mouse Unigene: 162368 Rat |
| 產(chǎn)品圖片 |
Paraformaldehyde-fixed, paraffin embedded (Mouse testis); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (GAA) Polyclonal Antibody, Unconjugated (bs-13254R) at 1:400 overnight at 4°C, followed by a conjugated Goat Anti-Rabbit IgG antibody (bs-0295G-FITC) for 90 minutes, and DAPI for nuclei staining.
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