• Species Reactivity

  Human

• Specificity

  Detects     in vitro synthesized CFTR and endogenous CFTR in Western blots.

• Source

  Monoclonal Mouse IgG    _2A Clone # 24-1

• Purification

  Protein A or G purified from hybridoma culture supernatant

• Immunogen

  Glutathione S-transferase-coupled CFTR    
  aa 1377-1480    
  Accession # P13569

• Formulation

  Supplied as a 0.2 μm filtered solution in PBS with BSA. *Small pack size (SP) is supplied as a 0.2 µm filtered solution in PBS.

• Label

  Unconjugated

Applications

• Recommended    
  Concentration

  Sample

• Western Blot

  1 µg/mL

  Human CFTR transfected cell line

• 
  

• Immunohistochemistry

  8-25 µg/mL

  See below

• 
  

• Immunoprecipitation

  1-2 µg/10    ⁶ cells

  T84 human colon carcinoma cell line,

• 
  

Please Note: Optimal dilutions should be determined by each laboratory for each application.  are available in the Technical Information section on our website.

Data Examples

Preparation and Storage

• Shipping

  The product is shipped with dry ice or equivalent. Upon receipt, store it immediately at the temperature recommended below. *Small pack size (SP) is shipped with polar packs. Upon receipt, store it immediately at -20 to -70 °C

• Stability & Storage

  Use a manual defrost freezer and avoid repeated freeze-thaw cycles.    

• 12 months from date of receipt, -20 to -70 °C, as supplied.

• 1 month, 2 to 8 °C under sterile conditions after opening.

• 6 months, -20 to -70 °C under sterile conditions after opening.

Background: CFTR

Cystic fibrosis transmembrane conductance regulator (CFTR) is a multi-pass transmembrane protein that functions as a chloride channel. CFTR belongs to the ATP‑binding cassette (ABC) superfamily. Mutations in CFTR cause the pulmonary disease, cystic fibrosis (CF). Specifically, deletion of phenyalanine at position 508 (DeltaF508-CFTR) results in a folding defect which impairs chloride channel function. The mechanism by which channel dysfunction relates to disease symptoms is a focus of intense research. CFTR dysfunction results in disruption of ion transport and subsequent blockage of airways by secreted mucus. CFTR may also play a role in the skeletal muscle atrophy and dysfunction that characterizes CF. In addition, CFTR-mediated chloride secretion underlies fluid accumulation and cyst growth in autosomal dominant polycystic kidney disease (ADPKD).

• Long Name:

  Cystic Fibrosis Transmembrane Conductance Regulator

• Entrez Gene IDs:

  1080 (Human)

• Alternate Names:

  ABC35; ABCC7CF; ATP-binding cassette sub-family C member 7; ATP-binding cassette transporter sub-family C member 7; cAMP-dependent chloride channel; CFTR; CFTR/MRP; Channel conductance-controlling ATPase; cystic fibrosis transmembrane conductance regulator (ATP-binding cassettesub-family C, member 7); cystic fibrosis transmembrane conductance regulator; cystic fibrosis transmembrane conductance regulator, ATP-binding cassette(sub-family C, member 7); dJ760C5.1; EC 3.6.3; MRP7EC 3.6.3.49; TNR-CFTR