CFTR Antibody

• 产品编号：CST-2269S      品牌：CST       原厂货号：2269S
• 产品分类：抗体 > 一抗 > 蛋白特异性一抗
• 应用分类：

描述：

该多克隆抗体用与人类CFTR蛋白氨基末端附近的氨基酸链对应的人工合成肽段免疫动物而制成。该抗体使用蛋白A和蛋白亲和层析纯化而得。CFTR（ABC35，ABCC7 CBAVD，CF，dj760C5.1，MRP7，TNR-CFTR）是ATP结合盒（ABC）转运蛋白超家族的成员。ABC基因突变与很多疾病相关。CFTR是质膜环磷酸腺苷激活的氯离子通道，在肺和其它器官的上皮细胞中表达（1,2）。它介导氯离子的分泌，还可以调节上皮钠通道（ENaC）、K+通道、ATP释放机制、阳离子交换子、钠碳酸氢盐转运体和水通道蛋白（3,4,5,6,7,8 9,10）。突变的CFTR基因导致囊性纤维化（CF），一种以胰腺外分泌功能不全、汗腺氯化钠增加、男性不育和呼吸道疾病为特点的疾病（1,2,11）。胞内运输会调节细胞表面CFTR分子的数量，从而间接部分调节Cl-分泌。苯丙氨酸508缺失(deltaF508)是CF病人最常见的突变。这种突变可导致在内质网滞留，因为内质网质量控制机制介导deltaF508突变体到蛋白酶体降解（12-15）。因此，破坏了CFTR转运会导致上皮细胞质膜氯离子分泌的失调。

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.

原厂资料：

Specificity / Sensitivity

CFTR Antibody detects endogenous levels of total CFTR protein.

Source / Purification

Polyclonal antibodies are produced by immunizing animals with a synthetic peptide corresponding to amino acids near the amino terminus of human CFTR. Antibodies are purified by protein A and peptide affinity chromatography.

Background

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.

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.

注意事项：

For Research Use Only. Not For Use In Diagnostic Procedures.

Storage: Supplied in 10 mM sodium HEPES (pH 7.5), 150 mM
NaCl, 100 µg/ml BSA and 50% glycerol. Store at –20°C. Do not
aliquot the antibody