Ca, cAMP & Lipid Signaling
| CSTコード |
包装 |
希望納入価格(円) |
国内在庫  |
ご登録代理店情報  カスタマー情報にご登録いただいた代理店を表示しています。
ご登録代理店の変更は こちら。 |
| #2269 | 100 μL | 46,000 | |
|
CFTR抗体製品一覧
推奨プロトコール
最適な結果を得るために:Cell Signaling Technology (CST) 社は、各製品の推奨プロトコールを使用することを強くお薦めいたします。
推奨プロトコールはCST社内試験の徹底的なバリデーションに基づいて作成されておりますので、正確かつ再現性の高い結果が得られます。
注:各製品に最適化されたプロトコールをリンクしています。
| | |
-
2269:
-
Western Blotting
| 用途(希釈倍率) | |
| ウェスタンブロッティング(1:1,000) |
| 特異性・感度 | |
| 内在性レベルのCFTR タンパク質を検出します。 |
| 使用抗原 | |
| ヒトのCFTR タンパク質のN末端近傍領域(合成ペプチド) |
Western Blotting
Western blot analysis of extracts of human airway epithelial cells, Calu-3 (expressing endogenous CFTR) and CFBE41o- (stably expressing WT-CFTR or deltaF508-CFTR), using CFTR Antibody. Kindly provided by Dr. Agnes Swiatecka-Urban and Dr. Bruce A. Stanton, Department of Physiology, Dartmouth Medical School, Hanover, NH.
Western Blotting
Western blot analysis of extracts from PANC-1 cells and whole mouse pancreas using CFTR Antibody.
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 (12-15). Therefore, disruption of CFTR trafficking leads to disregulation of Cl- secretion at the plasma membrane of epithelial cells.
-
Bradbury, N.A. et al. (1999)
Am. J. Physiol.
276, L659-L668.
-
Bertrand, C.A. and Frizzell, R.A. (2003)
Am. J. Physiol. Cell Physiol.
285, C1-C18.
-
Ko, S.B. et al. (2004)
Nat. Cell Biol.
6, 343-350.
-
Ji, H.L. et al. (2000)
J. Biol. Chem.
275, 27947-27956.
-
Jiang, Q. et al. (2000)
J. Biol. Chem.
275, 13266-13274.
-
Stutts, M.J. et al. (1997)
J. Biol. Chem.
272, 14037-14040.
-
Cheung, K.H. et al. (2003)
Biol. Reprod.
68, 1505-15010.
-
Shumaker, H. et al. (1999)
Am. J. Physiol.
276, C16-C25.
-
Schwiebert, E.M. et al. (1999)
Physiol. Rev.
79, S145-S166.
-
Yoo, D. et al. (2004)
J. Biol. Chem.
279, 6863-6873.
-
Cohn, J.A. et al. (2005)
Hum. Mutat.
26, 303-307.
-
Gibson, R.L. et al. (2003)
Am. J. Respir. Crit. Care Med.
168, 918-951.
-
Boucher, R.C. (2004)
Eur. Respir. J.
23, 146-158.
-
Riordan, J.R. (2005)
Annu. Rev. Physiol.
67, 701-718.