CFTR transports Cl- from cytosol to extracellular region

Stable Identifier
Reaction [transition]
Homo sapiens
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Cystic fibrosis transmembrane conductance regulator (CFTR) is a low conductance chloride-selective channel that mediates the transport of chloride ions in human airway epithelial cells which plays a key role in maintaining homoeostasis of epithelial secretions in the lungs. Defects in CFTR can cause cystic fibrosis (CF; MIM:602421), a common generalised disorder in Caucasians affecting the exocrine glands. CF results in an ionic imbalance that impairs clearance of secretions, not only in the lung, but also in the pancreas, gastrointestinal tract and liver (Riordan et al. 1999, Ousingsawat et al. 2011).

Literature References
PubMed ID Title Journal Year
2475911 Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA

Chou, JL, Alon, N, Kerem, B, Riordan, JR, Rozmahel, R, Plavsic, N, Grzelczak, Z, Lok, S, Zielenski, J, Rommens, JM

Science 1989
22178883 CFTR and TMEM16A are separate but functionally related Cl- channels

Ousingsawat, J, Kongsuphol, P, Kunzelmann, K, Schreiber, R

Cell. Physiol. Biochem. 2011
Catalyst Activity

chloride transmembrane transporter activity of CFTR [plasma membrane]

Orthologous Events
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