CFTR

CFTR (Cystic fibrosis transmembrane conductance regulator), mutations of which cause cystic fibrosis, belongs to the ATP-binding cassette (ABC) transporter family and works as a channel for small anions, such as chloride and bicarbonate. CFTR is composed of two homologous halves, each comprising a transmembrane (TMD) and a nucleotide binding domain (NBD). CFTR activity is regulated by phosphorylation of its cytosolic regulatory (R) domain, and ATP binding and hydrolysis at two NBDs.

CFTR is expressed in many cell types throughout the body, but in the airways it is found mainly in secretory serous cells of the submucosal glands. Transitions between open and closed states of CFTR are regulated by ATP binding and hydrolysis on the cytosolic nucleotide binding domains, which are coupled with the transmembrane (TM) domains forming the pathway for anion permeation. CFTR function is normally tightly controlled as dysregulation can lead to life-threatening diseases such as secretory diarrhoea and cystic fibrosis.

CFTR 相关产品（37）

货号	产品名	Cas
PL04947	(R)-BPO-27	1415390-47-4
PL02026	(R)-Olacaftor	1899111-41-1
PL01784	(R)-Posenacaftor sodium	2095064-09-6
PL02823	Bamocaftor	2204245-48-5
PL04948	BPO-27 racemate	1314873-02-3
PL01725	Cavosonstat	1371587-51-7
PL02756	CFTR corrector 2	1628416-28-3
PL03448	CFTR corrector 4	1918142-34-3
PL03645	CFTR corrector 6	2226970-01-8
PL04202	CFTR corrector 8	1918142-35-4
PL04213	CFTR corrector 9	909861-78-5
PC11934	CFTRinh-172	307510-92-5
PL01552	Crinecerfont hydrochloride	321839-75-2
PL01959	Elexacaftor	2216712-66-0
PL01872	Galicaftor	1918143-53-9
PL01870	GLPG1837	1654725-02-6
PL02414	GLPG2451	2055015-61-5
PL04382	Glyburide-d11	1189985-02-1
PL04383	Glyburide-d3	1219803-02-7
PL04799	GlyH-101	328541-79-3