Cystic Fibrosis Transmembrane Conductance Regulator (CFTR, ABCC7)

Cystic Fibrosis (CF) is a common lethal genetic disease caused by mutations of the gene coding for the cystic fibrosis transmembrane conductance factor, a cAMP regulated chloride channel. Approximately 70% of all CF cases share the deletion of a phenylalanine at position 508 (delta F508) which results in abnormal chloride transport. Since the CF mutation is lethal, most often by lung and liver disease, it raises the question of why this genetic disease remains as common as it is. One possible explanation is that Salmonella typhi has been shown to use CFTR to enter intestinal epithelial cells and that delta F508 heterozygote and homozygote mice showed 86% and 100% reductions in S. typhi intestinal submucosal uptake.\n\nApplications: \nSuitable for use in Immunofluorescence, Flow cytometry, Immunoprecipitation and Western Blot. Other applications not tested.\n\nRecommended Dilutions:\nImmunofluorescence: 1:500; Immunofluorescence staining of CFTR in mouse epithelial cells results in cell surface staining, consistent with localization at the plasma membrane.\nWestern Blot: 1:500; Detects a single ~170kD protein representing CFTR in T84 whole cell extract.\nOptimal dilutions to be determined by the researcher. \n\nStorage and Stability: \nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degrees C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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SPECIFICATIONS

Catalog Number

C9040-10

Size

100ul

Applications

FC, IF, IHC, IP, WB

Hosts

Mouse

Reactivities

Hum, Mouse

Form

Supplied as a liquid, 0.05% sodium azide.

P Type

Mab

Purity

Ascites

Isotype

IgM

References

1.Rottner M et al. FASEB J. 2007 Sep;21(11):2939-48. (WB: Hu, 1 ug/ml) 2. Pier GB et al. S Nature. 1998 May 7;393(6680):79-82. (WB: Ms) 3. Dalby-Payne JR et al. . Mol Biol Cell. 2003 Nov;14(11):4365-75. (WB: Hu; IHC: Hu) 4. Pier GB et al.. Nature. 1998 May 7;393(6680):79-82. (WB: Hu) 5. Walker J et al. J Cell Sci. 1995 Jun;108 ( Pt 6)():2433-44. (WB: Hu) 6. Laverty G et al. PTH stimulates a Cl(-)-dependent and EIPA-sensitive current in chick proximal tubule cells in culture. Am J Physiol Renal Physiol. 2003 May;284(5):F987-95. (WB: Ck) 7. Pier GB et al. Nature. 1998 May 7;393(6680):79-82. (Blocking: Hu, 1:10; Ms, 1:10) 8. Kulka M et al. J Pharmacol Exp Ther. 2005 Nov;315(2):563-70. (ICC: Rt, 1:50) 9. Kulka M et al. J Pharmacol Exp Ther. 2005 Nov;315(2):563-70. (ICC, Hu, 1:50) 10. Walker J et al. J Cell Sci. 1995 Jun;108 ( Pt 6)():2433-44. (ICC) 11. Kulka M, et al., J Leukoc Biol. 2002 Jan;71(1):54-64. (FACS: Hu, Rt; ICC: Rt, 1:100) 12. Benlloch, M., et al. J. Biol. Chem. 280(8): 6950-6959, 2005.

Additional Info

Recognizes human CTFR. Also detects one or more immunologically related proteins in murine cell line Heb7a that does not contain CFTR mRNA. Can also be used to inhibit the epithelial uptake of S. typhi in some mouse cell lines. The peptide sequence is highly conserved in mouse, sheep, bovine and Xenopus laevis. Species Crossreactivity: mouse, rat, chicken.