CFTR

CFTR

The ATP-binding cassette (ABC) transporter family includes the CFTR (Cystic Fibrosis Transmembrane Conductance Regulator), which functions as a channel for small anions like chloride and bicarbonate. Two identical halves of CFTR, each with a transmembrane (TMD) and a nucleotide binding domain (NBD), make up the entire structure. The cytosolic regulatory (R) domain of CFTR is phosphorylated, and ATP is bound and hydrolyzed at two NBDs to control CFTR activity.

Although CFTR is expressed in a wide variety of cell types throughout the body, it is primarily found in the secretory serous cells of the submucosal glands in the airways. The cytosolic nucleotide binding domains of CFTR are coupled with the transmembrane (TM) domains to form the pathway for anion permeation, and ATP binding and hydrolysis on these domains controls transitions between open and closed states. CFTR activity is typically tightly regulated because dysregulation can result in fatal conditions like cystic fibrosis and secretory diarrhoea.

CFTR related products

Structure Cat No. Product Name CAS No. Product Description
V74172 (R)-Elexacaftor ((R)-VX-445) 2229860-99-3
V74180 (R)-Olacaftor ((R)-VX-440) 1899111-41-1
V74181 (R)-Posenacaftor sodium ((R)-PTI-801 sodium) 2095064-09-6
V74179 (Rac)-Tezacaftor ((Rac)-VX-661) 1226709-85-8
V74163 Astressin 2B 681260-70-8
V74177 CFTR corrector 11 688050-45-5
V74164 CFTR corrector 12 958941-60-1
V74169 CFTR corrector 6 2226970-01-8
V74176 CFTR corrector 8 1918142-35-4
V74178 CFTRactivator 1 2768261-09-0
V74174 CP-628006 305822-08-6
V74165 CRF1 receptor antagonist-1 2635364-30-4
V10043 Elexacaftor (VX-445, WHO-11180) 2216712-66-0 Elexacaftor (VX-445, WHO-11180; WHO11180; VX445;Kaftrio;Trikafta)is one of three components in thefixed-dose combination medication (Elexacaftor/tezacaftor/ivacaftor, trade name: Trikafta and Kaftrio) used in patients who have cystic fibrosis with a F508del mutation.
V3551 FDL-169 1628416-28-3 FDL-169 is a novel and potent CFTR (Cystic fibrosis transmembrane conductance regulator) correctorbeing developed by Flatley Discovery Lab for treating cystic fibrosis (CF) patients who carry the F508del mutation, the most common mutation in this disease.
V4568 GLPG-1837 1654725-02-6 GLPG1837 (also known as ABBV-974) is a novel, potent and reversibleCFTR(cystic fibrosis transmembrane conductance regulator) potentiator withEC50s of 3 nM and 339 nM for F508del and G551D CFTR, respectively.
V20997 GlyH-101 328541-79-3 GlyH-101 is a novel and potent CFTR inhibitor (cystic fibrosis transmembrane conductance regulator) with Ki of 1.4 uM.
V3900 Ivacaftor benzenesulfonate (VX770) 1134822-09-5 Ivacaftor (also known as VX-770; trade name:KALYDECO andSymdeko) is a potent and orally active potentiator of CFTR (cystic fibrosis transmembrane conductance regulator) targeting G551D-CFTR and F508del-CFTR with EC50 of 100 nM and 25 nM in fisher rat thyroid cells, respectively.
V3901 Ivacaftor hydrate (VX-770) 1134822-07-3 Ivacaftor (also known as VX-770; trade name:KALYDECO andSymdeko) is a potent and orally bioactive potentiator of CFTR (cystic fibrosis transmembrane conductance regulator) targeting G551D-CFTR and F508del-CFTR with EC50 of 100 nM and 25 nM in fisher rat thyroid cells, respectively.
V76876 Ivacaftor-d4 (Ivacaftor-d4; VX-770-d4)
V76856 K41498 TFA
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