CFTR

The cystic fibrosis transmembrane conductance regulator (CFTR) is a 12-transmembrane ATP-binding cassette (ABC) transporter that functions as a chloride channel. It also regulates the activity of other channels including the epithelial sodium channel (ENaC).

Products
Background
Literature
Gene Data

Activators

Cat No Product Name / Activity
1422 DCEBIO
Activates Cl- conductance and hKCa3.1 channels

Blockers

Cat No Product Name / Activity
3430 CFTRinh 172
Voltage-independent, selective CFTR chloride channel blocker
1412 Chromanol 293B
Blocks ICFTR; also IKs blocker
0911 Glibenclamide
Blocks CFTR chloride channels; also KATP blocker
5485 GlyH 101
Reversible, voltage-dependent CFTR chloride channel blocker
1646 Lonidamine
CFTR Cl- channel blocker; also mitochondrial hexokinase inhibitor
4303 PPQ 102
Potent, voltage-independent CFTR chloride channel inhibitor

Other

Cat No Product Name / Activity
3143 KM 11060
Corrects F508del-CFTR trafficking
3943 PG 01
CFTR potentiator; corrects F508del-CFTR conductance defect

The cystic fibrosis transmembrane conductance regulator (CFTR) is a 12-transmembrane ATP-binding cassette (ABC) transporter that functions as a cAMP-dependent chloride channel. It is localized to the apical membrane of epithelial cells within the lungs, liver, pancreas, digestive tract, reproductive tract and skin, where it is involved in fluid transport.

CFTR is composed of five domains: two membrane-spanning domains (MSD1, MSD2) that function as a chloride channel; two nucleotide-binding domains (NBD1, NBD2) that possess ATPase activity; and a regulatory domain that contains a protein kinase A phosphorylation site, which controls channel opening. The CFTR may also regulate other channels including the epithelial sodium channel (ENaC) and the calcium-activated chloride channel (CaCC).

Mutations in the CFTR gene result in a number of diseases including cystic fibrosis (CF). The most common mutation detected in CF patients is a deletion of a phenylalanine residue within the NBD1 domain, and is known as the ΔF508 mutation. This results in a misfolded protein that is not trafficked to the apical membrane, but is instead retained in the endoplasmic reticulum where it undergoes proteasomal degradation. Absence of the CFTR at the apical membrane leads to an imbalance in intracellular chloride ion concentration, as well as the removal of the inhibitory actions of CFTR on ENaC function. This results in an increase in sodium ion conductance, inducing impaired transcellular water transport and abnormal lung secretions that promote bacterial infection.

Literature for CFTR

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  • 7-TM Receptors
  • Enzymes
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  • Transporters
  • Apoptosis
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  • Epigenetics
  • Fluorescent Imaging
  • Signal Transduction
  • Stem Cells

CFTR Gene Data

Gene Species Gene Symbol Gene Accession No. Protein Accession No.
CFTR Human CFTR NM_000492 P13569
Mouse Cftr NM_021050 P26361
Rat Cftr NM_031506 NP_113694