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- bs-16097R-FITC
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100ul
| 产品编号 | bs-16097R-FITC |
| 英文名称 | FKRP Rabbit pAb, FITC conjugated |
| 中文名称 | FITC标记的FKRP蛋白抗体 |
| 英文别名 | Fkrp; FKRP_HUMAN; FLJ12576; Fukutin related protein; Fukutin-related protein; LGMD2I; MDC1C; MGC2991. |
| 产品应用 | IF=1:100-500 Not yet tested in other applications. |
| 抗体来源 | Rabbit |
| 免疫原 | KLH conjugated synthetic peptide derived from human FKRP |
| 亚型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 克隆类型 | Polyclonal |
| 浓度 | 1mg/ml |
| 储存液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 研究领域 | Signal Transduction > Protein Trafficking > Golgi Proteins |
| 亚细胞定位 | Golgi apparatus. Secreted. Cell membrane > sarcolemma. Rough endoplasmic reticulum. According to some studies the N-terminal hydrophobic domain is cleaved after translocation to the Golgi apparatus and the protein is secreted. According to others the N-terminal hydrophobic domain is a transmembrane domain and the protein is a type II transmembrane type targeted to the Golgi apparatus by a non-cleavable signal anchor sequence. Localization at the cell membrane may require the presence of dystroglycan. At the Golgi apparatus localizes most likely at the cis-compartment. Detected in rough endoplasmic reticulum in myocytes. In general, mutants associated with severe clinical phenotypes are retained within the endoplasmic reticulum. |
| 组织特异性 | Expressed predominantly in skeletal muscle, placenta, and heart and relatively weakly in brain, lung, liver kidney and pancreas. |
| 翻译后修饰 | N-glycosylated. |
| 相似性 | Belongs to the licD transferase family. |
| 功能 | Could be a transferase involved in the modification of glycan moieties of alpha-dystroglycan (DAG1). |
| 保存条件 | Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 背景资料 | This gene encodes a protein which is targeted to the medial Golgi apparatus and is necessary for posttranslational modification of dystroglycan. Mutations in this gene have been associated with congenital muscular dystrophy, mental retardation, and cerebellar cysts. Several alternatively spliced transcript variants of this gene have been described, but the full-length nature of some of these variants has not been determined. [provided by RefSeq, Oct 2008] |
| 应用 | 推荐稀释比例 |
| {IF} | {1:100-500} |
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FKRP Rabbit pAb, FITC conjugated(bs-16097R-FITC)-100ul
￥2980
