验证数据展示
经过测试的应用
| Positive IF/ICC detected in | HepG2 cells |
推荐稀释比
| 应用 | 推荐稀释比 |
|---|---|
| Immunofluorescence (IF)/ICC | IF/ICC : 1:50-1:500 |
| It is recommended that this reagent should be titrated in each testing system to obtain optimal results. | |
| Sample-dependent, Check data in validation data gallery. | |
产品信息
CL594-66121 targets Alpha Galactosidase A in IF/ICC applications and shows reactivity with human samples.
| 经测试应用 | IF/ICC Application Description |
| 经测试反应性 | human |
| 免疫原 |
CatNo: Ag7505 Product name: Recombinant human GLA protein Source: e coli.-derived, PET28a Tag: 6*His Domain: 81-425 aa of BC002689 Sequence: WKDAGYEYLCIDDCWMAPQRDSEGRLQADPQRFPHGIRQLANYVHSKGLKLGIYADVGNKTCAGFPGSFGYYDIDAQTFADWGVDLLKFDGCYCDSLENLADGYKHMSLALNRTGRSIVYSCEWPLYMWPFQKPNYTEIRQYCNHWRNFADIDDSWKSIKSILDWTSFNQERIVDVAGPGGWNDPDMLVIGNFGLSWNQQVTQMALWAIMAAPLFMSNDLRHISPQAKALLQDKDVIAINQDPLGKQGYQLRQGDNFEVWERPLSGLAWAVAMINRQEIGGPRSYTIAVASLGKGVACNPACFITQLLPVKRKLGFYEWTSRLRSHINPTGTVLLQLENTMQMSL 种属同源性预测 |
| 宿主/亚型 | Mouse / IgG2a |
| 抗体类别 | Monoclonal |
| 产品类型 | Antibody |
| 全称 | galactosidase, alpha |
| 别名 | GLA, GALA, EC:3.2.1.22, Alpha-galactosidase A, Alpha-D-galactoside galactohydrolase |
| 计算分子量 | 49 kDa |
| 观测分子量 | 49 kDa |
| GenBank蛋白编号 | BC002689 |
| 基因名称 | GLA |
| Gene ID (NCBI) | 2717 |
| RRID | AB_2883517 |
| 偶联类型 | CoraLite®594 Fluorescent Dye |
| 最大激发/发射波长 | 588 nm / 604 nm |
| 形式 | Liquid |
| 纯化方式 | Protein A purification |
| UNIPROT ID | P06280 |
| 储存缓冲液 | PBS with 50% glycerol, 0.05% Proclin300, 0.5% BSA, pH 7.3. |
| 储存条件 | Store at -20°C. Avoid exposure to light. Stable for one year after shipment. Aliquoting is unnecessary for -20oC storage. |
背景介绍
GLA, also named as Melibiase and Alpha-galactosidase A, belongs to the glycosyl hydrolase 27 family. It hydrolyzes terminal, non-reducing alpha-D-galactose residues in alpha-D-galactosides, including galactose oligosaccharides, galactomannans and galactolipids. Fabry disease is an X-linked lysosomal storage disorder resulting from the deficient activity of GLA. Enzyme replacement therapy (ERT) with GLA is currently the most effective therapeutic strategy for patients with Fabry disease, a lysosomal storage disease.
实验方案
| Product Specific Protocols | |
|---|---|
| IF protocol for CL594 Alpha Galactosidase A antibody CL594-66121 | Download protocol |
| Standard Protocols | |
|---|---|
| Click here to view our Standard Protocols |
