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CTNS Antibody(C-term)
Affinity Purified Rabbit Polyclonal Antibody (Pab)
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Application 
| WB, E |
|---|---|
| Primary Accession | O60931 |
| Other Accession | NP_004928.2 |
| Reactivity | Human |
| Host | Rabbit |
| Clonality | Polyclonal |
| Isotype | Rabbit IgG |
| Calculated MW | 41738 Da |
| Antigen Region | 341-367 aa |
| Gene ID | 1497 |
|---|---|
| Other Names | Cystinosin, CTNS |
| Target/Specificity | This CTNS antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 341-367 amino acids from the C-terminal region of human CTNS. |
| Dilution | WB~~1:1000 E~~Use at an assay dependent concentration. |
| Format | Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification. |
| Storage | Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles. |
| Precautions | CTNS Antibody(C-term) is for research use only and not for use in diagnostic or therapeutic procedures. |
| Name | CTNS {ECO:0000303|PubMed:9537412, ECO:0000312|HGNC:HGNC:2518} |
|---|---|
| Function | Cystine/H(+) symporter that mediates export of cystine, the oxidized dimer of cysteine, from lysosomes (PubMed:11689434, PubMed:15128704, PubMed:18337546, PubMed:22232659, PubMed:29467429, PubMed:33208952, PubMed:36113465). Plays an important role in melanin synthesis by catalyzing cystine export from melanosomes, possibly by inhibiting pheomelanin synthesis (PubMed:22649030). In addition to cystine export, also acts as a positive regulator of mTORC1 signaling in kidney proximal tubular cells, via interactions with components of the v-ATPase and Ragulator complexes (PubMed:36113465). Also involved in small GTPase-regulated vesicle trafficking and lysosomal localization of LAMP2A, independently of cystine transporter activity (By similarity). |
| Cellular Location | [Isoform 1]: Lysosome membrane; Multi-pass membrane protein. Melanosome membrane; Multi-pass membrane protein. Note=AP-3 complex is required for localization to the lysosome. |
| Tissue Location | Strongly expressed in pancreas, kidney (adult and fetal), skeletal muscle, melanocytes and keratinocytes (PubMed:22649030). Expressed at lower levels in placenta and heart Weakly expressed in lung, liver and brain (adult and fetal) (PubMed:22649030). |
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Provided below are standard protocols that you may find useful for product applications.
Background
This gene encodes a seven-transmembrane domain protein that functions to transport cystine out of lysosomes. Its activity is driven by the H+ electrochemical gradient of the lysosomal membrane. Mutations in this gene cause cystinosis, a lysosomal storage disorder. Alternative splicing results in multiple transcript variants.
References
Taranta, A., et al. Pediatr. Nephrol. 25(7):1263-1267(2010)
Bellomo, F., et al. Free Radic. Biol. Med. 48(7):865-872(2010)
Kumar, A., et al. J. Biosci. 35(1):21-25(2010)
Aldahmesh, M.A., et al. Ophthalmic Genet. 30(4):185-189(2009)
Macias-Vidal, J., et al. Clin. Genet. 76(5):486-489(2009)
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