验证数据展示
经过测试的应用
| Positive WB detected in | mouse skeletal muscle tissue, mouse brain tissue, rat skeletal muscle tissue |
| Positive IHC detected in | human ovary cancer tissue Note: suggested antigen retrieval with TE buffer pH 9.0; (*) Alternatively, antigen retrieval may be performed with citrate buffer pH 6.0 |
| Positive IF/ICC detected in | HEK-293 cells |
推荐稀释比
| 应用 | 推荐稀释比 |
|---|---|
| Western Blot (WB) | WB : 1:500-1:2000 |
| Immunohistochemistry (IHC) | IHC : 1:50-1:500 |
| 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
15094-1-AP targets GMPPB in WB, IHC, IF/ICC, ELISA applications and shows reactivity with human, mouse, rat samples.
| 经测试应用 | WB, IHC, IF/ICC, ELISA Application Description |
| 文献引用应用 | WB, IHC, IF |
| 经测试反应性 | human, mouse, rat |
| 文献引用反应性 | human, mouse, zebrafish |
| 免疫原 | GMPPB fusion protein Ag7168 种属同源性预测 |
| 宿主/亚型 | Rabbit / IgG |
| 抗体类别 | Polyclonal |
| 产品类型 | Antibody |
| 全称 | GDP-mannose pyrophosphorylase B |
| 别名 | Mannose-1-phosphate guanyltransferase beta, KIAA1851, GTP-mannose-1-phosphate guanylyltransferase beta, GDP-mannose pyrophosphorylase B, EC:2.7.7.13 |
| 计算分子量 | 40 kDa |
| 观测分子量 | 40-43 kDa |
| GenBank蛋白编号 | BC001141 |
| 基因名称 | GMPPB |
| Gene ID (NCBI) | 29925 |
| RRID | AB_2111064 |
| 偶联类型 | Unconjugated |
| 形式 | Liquid |
| 纯化方式 | Antigen affinity purification |
| UNIPROT ID | Q9Y5P6 |
| 储存缓冲液 | PBS with 0.02% sodium azide and 50% glycerol , pH 7.3 |
| 储存条件 | Store at -20°C. Stable for one year after shipment. Aliquoting is unnecessary for -20oC storage. |
背景介绍
GMPPB is a cytoplasmic enzyme that catalyzes the formation of GDP-mannose, a key substrate for several glycosylation pathways, including O-mannosylation of α-DG. GMPPB facilitates the formation of the sugar donor GDP-mannose and pyrophosphate from mannose-1-phosphate and GTP, a very early step in the glycosylation pathway (PMID: 37834154, 27147698). GMPPB mutations correlate with several congenital diseases, including severe congenital muscular dystrophy (CMD) with abnormalities in the brain and eye (PMID: 26310427, 30684953). Western blot analysis detected GMPPB at an apparent molecular mass of 40 kDa.
实验方案
| Product Specific Protocols | |
|---|---|
| WB protocol for GMPPB antibody 15094-1-AP | Download protocol |
| IHC protocol for GMPPB antibody 15094-1-AP | Download protocol |
| IF protocol for GMPPB antibody 15094-1-AP | Download protocol |
| Standard Protocols | |
|---|---|
| Click here to view our Standard Protocols |
发表文章
| Species | Application | Title |
|---|---|---|
iScience A Trace Amount of Galactose, a Major Component of Milk Sugar, Allows Maturation of Glycoproteins during Sugar Starvation. | ||
Mol Biomed GMPPB-congenital disorders of glycosylation associate with decreased enzymatic activity of GMPPB. | ||
Front Nutr Altered mannose metabolism in chronic stress and depression is rapidly reversed by vitamin B12 | ||
Int J Mol Sci Silencing GMPPB Inhibits the Proliferation and Invasion of GBM via Hippo/MMP3 Pathways
| ||
Front Mol Neurosci Consequences of GMPPB deficiency for neuromuscular development and maintenance |
