Reactome | CFTR transports Cl- from cytosol to extracellular region

CFTR transports Cl- from cytosol to extracellular region

Stable Identifier

R-HSA-5678863

Type

Reaction [transition]

Species

Homo sapiens

Compartment

cytosol, extracellular region, plasma membrane

ReviewStatus

5/5

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CFTR transports Cl- from cytosol to extracellular region

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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