| 货号 | 78335S |
| 同种亚型 | Rabbit IgG |
| 反应种属 | Human |
| 应用 | WB,IP,IF |
| 目标/特异性 | CFTR (D6W6L) Rabbit mAb recognizes endogenous levels of total CFTR protein. This antibody also detects a 60 kDa band of unknown origin in some cell lines. |
| 使用方法 | Western Blotting (1:1000) Immunoprecipitation (1:50) Immunofluorescence (Immunocytochemistry) (1:800) |
| 供应商 | CST |
| 灵敏度 | Endogenous |
| 背景 | CFTR (ABC35, ABCC7, CBAVD, CF, dj760C5.1, MRP7, TNR-CFTR) is a member of the ATP-binding cassette (ABC) transporter superfamily. Mutations in ABC genes have been linked to many diseases. CFTR is a plasma membrane cyclic AMP activated chloride channel that is expressed in the epithelial cells of the lung and several other organs (1,2). It mediates the secretion of Cl- and also regulates several channels including the epithelial sodium channel (ENaC), K+ channels , ATP release mechanisms, anion exchangers, sodium bicarbonate transporters and aquaporin water channels (3,4,5,6,7,8 9,10). Mutations in the CFTR gene cause cystic fibrosis, a disease that is characterized by exocrine pancreatic insufficiency, increase in sweat gland NaCl, male infertility and airway disease (1,2,11). Intracellular trafficking regulates the number of CFTR molecules at the cell surface, which in part regulates Cl- secretion. Deletion of phenylalanine 508 (deltaF508) is the most common mutation in CF patients. This mutation results in retention in the ER, where ER quality control mechanisms target the deltaF508 mutant to the proteosome for degradation (12-14). Therefore, disruption of CFTR trafficking leads to disregulation of Cl- secretion at the plasma membrane of epithelial cells. |
| 存放说明 | -20C |
| 计算分子量 | 150-220 |
| 参考文献 | 1 . Bradbury, N.A. et al. (1999) Am. J. Physiol. 276, L659-L668. 2 . Bertrand, C.A. and Frizzell, R.A. (2003) Am. J. Physiol. Cell Physiol. 285, C1-C18. 3 . Ko, S.B. et al. (2004) Nat. Cell Biol. 6, 343-350. 4 . Ji, H.L. et al. (2000) J. Biol. Chem. 275, 27947-27956. 5 . Jiang, Q. et al. (2000) J. Biol. Chem. 275, 13266-13274. 6 . Stutts, M.J. et al. (1997) J. Biol. Chem. 272, 14037-14040. 7 . Cheung, K.H. et al. (2003) Biol. Reprod. 68, 1505-15010. 8 . Shumaker, H. et al. (1999) Am. J. Physiol. 276, C16-C25. 9 . Schwiebert, E.M. et al. (1999) Physiol. Rev. 79, S145-S166. 10 . Yoo, D. et al. (2004) J. Biol. Chem. 279, 6863-6873. 11 . Cohn, J.A. et al. (2005) Hum. Mutat. 26, 303-307. 12 . Gibson, R.L. et al. (2003) Am. J. Respir. Crit. Care Med. 168, 918-951. 13 . Boucher, R.C. (2004) Eur. Respir. J. 23, 146-158. 14 . Riordan, J.R. (2005) Annu. Rev. Physiol. 67, 701-718. |
Western blot analysis of extracts from Calu-3, U-2 OS, and HT-29 cells, using CFTR (D6W6L) Rabbit mAb and β-Actin (D6A8) Rabbit mAb #8457. | |
Immunoprecipitation of CFTR from HT-29 cell extracts. Lane 1 is 10% input, lane 2 is Rabbit (DA1E) mAb IgG XP® Isotype Control #3900, and lane 3 is CFTR (D6W6L) Rabbit mAb. Western blot analysis was performed using CFTR (D6W6L) Rabbit mAb. | |
Confocal immunofluorescent analysis of Calu-3 (left, positive) and U-2 OS (right, negative) cells using CFTR (D6W6L) Rabbit mAb (green). Blue pseudocolor = DRAQ5® #4084 (fluorescent DNA dye). |
