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Render Timestamp: 2024-10-09T09:54:20.963Z
Commit: f04ddd7fea9fb3592f59f61482fcb94610d25cbe
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PDP - Template Name: Monoclonal Antibody
PDP - Template ID: *******c5e4b77
R Recombinant
Recombinant: Superior lot-to-lot consistency, continuous supply, and animal-free manufacturing.

CFTR (D6W6L) Rabbit mAb #78335

Filter:
  • WB
  • IP
  • IF

    Supporting Data

    REACTIVITY H
    SENSITIVITY Endogenous
    MW (kDa) 150-220
    Source/Isotype Rabbit IgG
    Application Key:
    • WB-Western Blotting 
    • IP-Immunoprecipitation 
    • IF-Immunofluorescence 
    Species Cross-Reactivity Key:
    • H-Human 

    Product Information

    Product Usage Information

    Application Dilution
    Western Blotting 1:1000
    Immunoprecipitation 1:50
    Immunofluorescence (Immunocytochemistry) 1:400 - 1:1600

    Storage

    Supplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg/ml BSA, 50% glycerol and less than 0.02% sodium azide. Store at –20°C. Do not aliquot the antibody.

    For a carrier free (BSA and azide free) version of this product see product #30167.

    Protocol

    Specificity / Sensitivity

    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.

    Species Reactivity:

    Human

    Source / Purification

    Monoclonal antibody is produced by immunizing animals with recombinant protein fragment of human CFTR protein. The epitope corresponds to a region surrounding Arg735 of human CFTR.

    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 (12-14). 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.
    For Research Use Only. Not For Use In Diagnostic Procedures.
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