Anti-CFTR Antibody

Cystic fibrosis transmembrane conductance regulator, ATP-binding cassette sub-family C member 7, ABCC7, cAMP-dependent Cl- channel
    Cat #: ACL-006
    Alternative Name Cystic fibrosis transmembrane conductance regulator, ATP-binding cassette sub-family C member 7, ABCC7, cAMP-dependent Cl- channel
  • KO Validated
  • Lyophilized Powder
  • Antigen Incl.
  • Type: Polyclonal
    Host: Rabbit
    Reactivity: h, m, r
      • Peptide (C)KEETEEEVQDTRL, corresponding to amino acid residues 1468-1480 of human CFTR (Accession P13569). Cytoplasmic, C-terminal part.
        Anti-CFTR Antibody
    Accession (Uniprot) Number P13569
    Gene ID 1080
    Peptide confirmation Confirmed by amino acid analysis and mass spectrometry.
    Homology Mouse, rat, pig - 13/14 amino acid residues identical.
    RRID AB_2039804.
    Purity Affinity purified on immobilized antigen.
    Form Lyophilized powder. Reconstituted antibody contains phosphate buffered saline (PBS), pH 7.4, 1% BSA, 0.05% NaN3.
    Isotype Rabbit IgG.
    Storage before reconstitution The antibody ships as a lyophilized powder at room temperature. Upon arrival, it should be stored at -20°C.
    Reconstitution 25 μl, 50 μl or 0.2 ml double distilled water (DDW), depending on the sample size.
    Antibody concentration after reconstitution 0.8 mg/ml.
    Storage after reconstitution The reconstituted solution can be stored at 4°C for up to 1 week. For longer periods, small aliquots should be stored at -20°C. Avoid multiple freezing and thawing. Centrifuge all antibody preparations before use (10000 x g 5 min).
    Negative control antigen storage before reconstitution Lyophilized powder can be stored intact at room temperature for 2 weeks. For longer periods, it should be stored at -20°C.
    Negative control antigen reconstitution 100 µl double distilled water (DDW).
    Negative control antigen storage after reconstitution -20°C.
    Preadsorption Control 1 μg peptide per 1 μg antibody.
    Standard quality control of each lot Western blot analysis.
    Applications: ic, if, ih, ip, wb
    May also work in: ifc*
      • Anti-CFTR Antibody
        Western blot analysis of rat lung membranes:
        1. Anti-CFTR Antibody (#ACL-006), (1:200).
        2. Anti-CFTR Antibody, preincubated with the control peptide antigen.
        Mouse caveolar fraction lysate (1:500) (Tabeling, C. et al. (2015) Proc. Natl. Acad. Sci. U.S.A. 112, E1614.)
      • Mouse embryonic stem cells (Liu, Z. et al. (2017) Cell Death Differ. 24, 98.).
      • Anti-CFTR Antibody
        Expression of CFTR in rat lungs
        Immunohistochemical staining of rat lungs sections using Anti-CFTR Antibody (#ACL-006) (left panel). Strong staining of bronchial epithelial cells (red) and lighter staining of alveolar cells (red-brown) is apparent. There is also positive staining of macrophages while smooth muscle and endothelium are negative. Counterstain of cell nuclei appears blue. A negative control is shown in the right panel.
      • Mouse embryonic stem cells (Liu, Z. et al. (2017) Cell Death Differ. 24, 98.).
    References
      • The cystic fibrosis transmembrane conductance regulator (CFTR) is the most dominant Cl- channel in several epithelial tissues, especially in lung and colon. Remarkably, CFTR is a member of the ATP-binding cassette (ABC) transporter superfamily that uses ATP hydrolyzation as the driving force for the translocation of a wide variety of substrates including sugars, amino acids, proteins and hydrophobic compounds, across cellular membranes. The CFTR is unique among ABC transporters in that it is a cAMP-regulated Cl- channel. It shares the superfamily topology of 12 transmembrane domains with two nucleotide-binding domains (NBDs) and a regulatory (R) domain in the large third intracytoplasmic loop that is phosphorylated in multiple sites by PKA. Mutations in the CFTR gene cause channel dysfunction in several ways, ranging from complete loss of surface expression to diminished Cl- secretion.  Defects in the CFTR gene cause cystic fibrosis (CF), the most common genetic disease among Caucasians, as well as a form of male sterility.

        Regulation of the CFTR channel is accomplished through the activation of surface receptors that couple to adenyl cyclase, raise cAMP cellular levels and thus activate PKA. This has been demonstrated for the adenosine and ß2 adrenergic receptor and the vasopressin hormone among others.

        Besides enhanced Cl- conductance, activation of CFTR also leads to the regulation of other ion channels. The best-studied case is its interaction with the epithelial Na+ channels (ENaC), although it can probably regulate other ion channels as well (Kir1.1 for example). The mechanism by which CFTR regulates other ion channels is not clear, but it may involve protein-protein interactions via molecules that interact with its C-terminal PDZ binding motif, such as the NHERF adaptor protein.

    Application key:

    CBE- Cell-based ELISA, FC- Flow cytometry, ICC- Immunocytochemistry, IE- Indirect ELISA, IF- Immunofluorescence, IFC- Indirect flow cytometry, IHC- Immunohistochemistry, IP- Immunoprecipitation, LCI- Live cell imaging, N- Neutralization, WB- Western blot

    Species reactivity key:

    H- Human, M- Mouse, R- Rat
    Image & Title:

    Anti-CFTR Antibody
    Knockout validation of Anti-CFTR Antibody in mouse pancreas.Immunohistochemical staining of mouse pancreas sections using Anti-CFTR Antibody (#ACL-006). CFTR immunoreactivity (red) is exclusively localized to apical membranes in the ducts. AQP1 staining (green) is observed throughout the plasma membrane. CFTR is not detected in CFTR-/- mice (bottom pannels).Adapted from Venglovecz, V. et al. (2018) Front. Physiol. 9, 854. with permission of Frontiers.

    Last update: 24/01/2020

    Anti-CFTR Antibody (#ACL-006) is a highly specific antibody directed against an epitope of the human protein. The antibody can be used in western blot, immunoprecipitation, immunohistochemistry, and immunocytochemistry applications. It has been designed to recognize CFTR from mouse, rat, and human samples.

    For research purposes only, not for human use
    Citations
      • Anti-CFTR Antibody
        Expression of CFTR in human Caco-2 cells
        Immunocytochemical staining of human epithelial colorectal adenocarcinoma cells using Anti-CFTR Antibody (#ACL-006). CFTR (green) co-localizes with β-Catenin (red). To-Pro3 is used to stain nuclei.
        Adapted from Liu, K. et al. (2016) Oncotarget 7, 64030. with permission of Impact Journals.
      • Immunohistochemical staining of mouse pancreas sections. Tested in CFTR-/- mice.
        Venglovecz, V. et al. (2018) Front. Physiol. 9, 854.
      • Mouse embryonic stem cells.
        Liu, Z. et al. (2017) Cell Death Differ. 24, 98.
      • Rat ovary lysates (1:200).
        Chen, H. et al. (2015) Reproduction 149, 393.
      • Mouse caveolar fraction lysate (1:500).
        Tabeling, C. et al. (2015) Proc. Natl. Acad. Sci. U.S.A. 112, E1614.
      • Mouse uterus lysate (1:1200).
        Zhou, M. et al. (2014) PLoS ONE 9, e99521.
      • Mouse embryonic stem cells.
        Liu, Z. et al. (2017) Cell Death Differ. 24, 98.
      • Mouse pancreas sections. Also tested in CFTR-/- mice.
        Venglovecz, V. et al. (2018) Front. Physiol. 9, 854.
      • Mouse tendon sections.
        Liu, Y. et al. (2017) FASEB J. 31, 3800.
      • Rat ovary sections (1:50).
        Chen, H. et al. (2015) Reproduction 149, 393.
      • Mouse uterus sections (1:1000).
        Zhou, M. et al. (2014) PLoS ONE 9, e99521.
      • Mouse lung sections (1:500).
        Li, S. and Xiang, M. (2011) Dev. Dyn. 240, 1512.
      • Mouse embryonic stem cells.
        Liu, Z. et al. (2017) Cell Death Differ. 24, 98.
      • Human epithelial colorectal adenocarcinoma cells (Caco-2).
        Liu, K. et al. (2016) Oncotarget 7, 64030.
      • Chen, J. et al. (2012) J. Cell. Physiol. 227, 2759.
      • Merigo, F. et al. (2011) Dev. Neurobiol. 71, 854.
      • Sorio, C. et al. (2011) PLoS One 6, e22212.
      • Chen, M.H. et al. (2010) Hum. Reprod. 25, 1744.
      • Merigo, F. et al. (2009) Cell Tissue Res. 336, 411.
      • Chen, M. et al. (2008) Acta Biochim. Biophys. Sin. 40, 864.
      • Ishibashi, K. et al. (2008) Am. J. Physiol. Regul. Integr. Comp. Physiol. 294, R1729.
      • Merigo, F. et al. (2008) Chem. Senses 33, 231.
      • Pietrement, C. et al. (2008) J. Biol. Chem. 283, 2986.
      • Li, J. et al. (2007) J. Biol. Chem. 282, 27086.
      • Xu, W.M. et al. (2007) Proc. Natl. Acad. Sci. USA 104, 9816.
      • Ishibashi, K. et al. (2006) J. Dent. Res. 85, 1101.
      • Li, J. et al. (2005) J. Biol. Chem. 45, 37634.
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