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Product Usage Information

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.

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.

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.

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.

Entrez-Gene Id 1080
Swiss-Prot Acc. P13569

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