- ￥880
- Bioss
- bs-12932P
- 2025年10月16日
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- 详细信息
- 技术资料
- 规格：
500ug
| 产品编号 | bs-12932P |
| 英文名称 | CTNS Antibody Blocking Peptide |
| 中文名称 | 胱氨酸封闭多肽 |
| 英文别名 | CTNS LSB; Cystinosin; cystinosis, nephropathic; PQLC4; CTNS_HUMAN. |
| 纯化方法 | HPLC |
| 研究领域 | Metabolism > Pathways and Processes > Metabolic signaling pathways > Amino acid metabolism Signal Transduction > Metabolism > Amino Acids Tags & Cell Markers > Subcellular Markers > Organelles > Lysosome |
| 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). |
| 相似性 | Belongs to the cystinosin family. Contains 2 PQ-loop domains. |
| 功能 | CTNS (Cystinosin) is thought to transport cystine out of lysosomes. Mutations in the CTNS gene are the cause of cystinosis. |
| 保存条件 | Shipped at 4℃. Stored at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 背景资料 | 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. |
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CTNS Antibody Blocking Peptide(bs-12932P)-500ug
￥880
