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Anti-CTNS Polyclonal Antibody 是一种 Rabbit 抗体,靶向 CTNS。Anti-CTNS Polyclonal Antibody 可用于 WB。
Anti-CTNS Polyclonal Antibody 是一种 Rabbit 抗体,靶向 CTNS。Anti-CTNS Polyclonal Antibody 可用于 WB。
| 规格 | 价格 | 库存 | 数量 |
|---|---|---|---|
| 50 μL | ¥ 1,170 | 5日内发货 | |
| 100 μL | ¥ 1,965 | 5日内发货 | |
| 200 μL | ¥ 2,785 | 5日内发货 |
| 产品描述 | Anti-CTNS Polyclonal Antibody is a Rabbit antibody targeting CTNS. Anti-CTNS Polyclonal Antibody can be used in WB. |
| Ig Type | IgG |
| 反应种属 | Human,Mouse,Rat (predicted:Rabbit) |
| 应用 | WB |
| 推荐剂量 | WB: 1:500-2000 |
| 抗体种类 | Polyclonal |
| 宿主来源 | Rabbit |
| 亚细胞定位 | Lysosome membrane; Multi-pass membrane protein. |
| 组织特异性 | Strongly expressed in pancreas, kidney (adult and fetal) and in skeletal muscle. Expressed at lower levels in placenta and heart. Weakly expressed in lung, liver and brain (adult and fetal). |
| 构建方式 | Hybridoma Polyclonal Antibody |
| 纯化方式 | Protein A purified |
| 性状 | Liquid |
| 缓冲液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 浓度 | 1mg/ml |
| 研究背景 | Cystinosis is an autosomal recessive disorder resulting from defective lysosomal transport of cystine and present at birth as a failure to thrive, rickets and proximal renal tubular acidosis. The human CTNS gene on chromosome 17p13 encodes the protein Cystinosin, and mutations in CTNS are responsible for nephropathic cystinosis. The CTNS promoter contains an Sp1 binding element. Cystinosin is an integral membrane protein containing 7 transmembrane domains that functions as a H+-driven transporter responsible for cystine export from lysosomes. In humans, Cystinosin is expressed abundantly in pancreas, kidney (mature and fetal), and skeletal muscle. The mouse homolog to CTNS encodes a protein which is expressed in all tissues except skeletal muscle. In the cell, Cystinosin co-localizes with LAMP-2 to lysosomes. A C-terminal GYDQL sorting motif within Cystinosin is critical for lysosomal localization. |
| 免疫原 | KLH conjugated synthetic peptide: human CTNS/Cystinosin |
| 抗原种属 | Human |
| 基因名称 | CTNS |
| 基因ID | |
| 蛋白名称 | Cystinosin |
| Uniprot ID | |
| 功能 | CTNS (Cystinosin) is thought to transport cystine out of lysosomes. Mutations in the CTNS gene are the cause of cystinosis. |
| 分子量 | Theoretical: 42 kDa. |
| 储存方式 | Store at 2°C-8°C for 1 month. Store at -20°C or -80°C for 12 months. Avoid repeated freeze-thaw cycles. |
| 运输方式 | Shipping with blue ice. |
| 存储 | store at low temperature | -20°C for 1 year |