

| Catalog No. | HF632014 | ||||||||
|---|---|---|---|---|---|---|---|---|---|
| Species reactivity | Human | ||||||||
| Applications | ELISA, IHC, WB | ||||||||
| Host species | Rabbit | ||||||||
| Isotype | IgG | ||||||||
| Clonality | Polyclonal | ||||||||
| Immunogen | E. coli - derived recombinant Human CTNS (Ser22-Ser120). | ||||||||
| Target | CTNS, Cystinosin | ||||||||
| Purification | Purified by antigen affinity column. | ||||||||
| Accession | O60931 | ||||||||
| Form | Liquid | ||||||||
| Storage buffer | 0.01M PBS, pH 7.4, 50% Glycerol, 0.05% Proclin 300. Please refer to the specific buffer information in the hardcopy of datasheet or the lot-specific COA. |
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| Product Usage Information |
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| Stability and Storage | Use a manual defrost freezer and avoid repeated freeze thaw cycles. Store at 2 to 8°C for frequent use. Store at -20 to -80°C for twelve months from the date of receipt. | ||||||||
| Background | Cystinosin (CTNS) is a ~41 kDa protein. Cystine/H(+) symporter that mediates export of cystine, the oxidized dimer of cysteine, from lysosomes. Plays an important role in melanin synthesis by catalyzing cystine export from melanosomes, possibly by inhibiting pheomelanin synthesis. 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. Also involved in small GTPase-regulated vesicle trafficking and lysosomal localization of LAMP2A, independently of cystine transporter activity. 1. Kalatzis, V. et al. (2001) The EMBO journal 20, 5940-9. PMID: 11689434 2. Kalatzis, V. et al. (2004) Human molecular genetics 13, 1361-71. PMID: 15128704 3. Taranta, A. et al. (2008) American journal of physiology. Renal physiology 294, F1101-8. PMID: 18337546 4. Ruivo, R. et al. (2012) Proceedings of the National Academy of Sciences of the United States of America 109, E210-7. PMID: 22232659 5. Deshpande, AA. et al. (2018) Scientific reports 8, 3442. PMID: 29467429 6. Adelmann, CH. et al. (2020) Nature 588, 699-704. PMID: 33208952 7. Guo, X. et al. (2022) Cell 185, 3739-3752.e18. PMID: 36113465 8. Chiaverini, C. et al. (2012) FASEB journal : official publication of the Federation of American Societies for Experimental Biology 26, 3779-89. PMID: 22649030 | ||||||||
| Note | For research use only. |
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