CFTR

シグナル伝達経路

研究分野

阻害剤の選択性比較

カタログ番号	製品カタログ	溶解度(25°C)
カタログ番号	製品カタログ	水	DMSO	アルコール
S6003	Ataluren (PTC124)	<1 mg/mL	57 mg/mL	<1 mg/mL
S7139	CFTRinh-172	<1 mg/mL	82 mg/mL	<1 mg/mL
S7329	IOWH032	<1 mg/mL	100 mg/mL	<1 mg/mL
S8094	GlyH-101	<1 mg/mL	98 mg/mL	<1 mg/mL
S1144	Ivacaftor (VX-770)	<1 mg/mL	78 mg/mL	<1 mg/mL
S8698	GLPG1837	<1 mg/mL	69 mg/mL	5 mg/mL
S1565	VX-809 (Lumacaftor)	<1 mg/mL	90 mg/mL	6 mg/mL
S7059	Tezacaftor (VX-661)	<1 mg/mL	100 mg/mL	<1 mg/mL
S8795	FDL169	<1 mg/mL	33 mg/mL	8 mg/mL

CFTR製品

All (9) CFTR阻害剤(4) CFTR活性剤(2) CFTRモジュレータ(3) 新CFTR製品

製品コード	製品説明	文献中Selleckの製品使用例	お客様のフィードバック
S6003	Ataluren (PTC124) Ataluren (PTC124) selectively induces ribosomal read-through of premature but not normal termination codons, with EC50 of 0.1 μM in HEK293 cells, may provide treatment for genetic disorders caused by nonsense mutations (e.g. CF caused by CFTR nonsense mutation). Phase 3.	Haematologica, 2019, 10.3324/haematol.2018.212118 J Cancer, 2019, 10(2):287-292 Am J Hematol, 2018, 93(4):527-536	Effect of systemic ataluren treatment on mice with the Pax6^Sey+/- phenotype. The black arrowhead indicates the lenticular stalk; the black arrow indicates the cornea; and the asterisk indicates the ciliary margin. WT, Pax6^+/+; Mt, Pax6^Sey/+; L, lens; r, retina. Original magnification, x5.
S7139	CFTRinh-172 CFTRinh-172 is a voltage-independent, selective CFTR inhibitor with K_i of 300 nM, showing no effects on MDR1, ATP-sensitive K+ channels, or a series of other transporters.	Nature, 2019, 567(7748):405-408 Int J Mol Med, 2018, 41(6):3501-3508 Reproduction, 2018, 156(3):261-268	CFTR inhibitor CFTR_inh172 leads to PGCs disorder in early zebrafish embryo. Analysis of localization of nanos1/vasa positive cells in embryos by WISH at 50% Epiboly stage with top view.
S7329	IOWH032 IOWH032 is a synthetic CFTR inhibitor with IC50 of 1.01 μM in CHO-CFTR cell based assays. Phase 2.
S8094	GlyH-101 GlyH-101 is a selective and reversible CFTR inhibitor with K_i of 4.3 μM.
S1144	Ivacaftor (VX-770) Ivacaftor (VX-770) is a selective potentiator of CFTR targeting G551D-CFTR and F508del-CFTR with EC50 of 100 nM and 25 nM in fisher rat thyroid cells, respectively.	EMBO J, 2019, 38(4) Cell Rep, 2019, 26(7):1701-1708 J Control Release, 2019, 296:225-231	(A) CFTR Western blot of normal (NL) and CF HBE cultures treated with VX-809 (5 uM) ± VX-770 (5 uM) for 48 hours. “*” indicates the mature, complex glycosylated form of CFTR, band C; “•” indicates the immature band B. (B) Turnover of rescued ΔF508 in BHK-21 cells. ΔF508 was rescued at 27°C in the presence of VX-809 ± VX-770 for 24 hours. After adding cycloheximide (200 ug/ml, 37°C), cells were lysed at the indicated times and analyzed by Western blotting.
S8698	GLPG1837 GLPG1837 is a novel CFTR potentiator with an EC50 value of 3 nM for F508del, showing enhanced efficacy on CFTR mutants harboring class III mutations compared to Ivacaftor.
S1565	VX-809 (Lumacaftor) VX-809 (Lumacaftor) acts to correct CFTR mutations common in cystic fibrosis by increasing mutant CFTR (F508del-CFTR) maturation,EC50 of 0.1 μM in fisher rat thyroid cells. Phase 3.	Am J Respir Crit Care Med, 2019, 199(1):123-126 EMBO J, 2019, 38(4) Cell Rep, 2019, 26(7):1701-1708	△F508CFTR diffusion is increased by different correction mechanisms. A, example trajectories from MDCK cell lines for wtCFTR (black) and △F508CFTR rescued by VX-809 treatment in the absence (red) and presence (blue ) of mCh-CFTR-C. B, summary graph for wtCFTR, r△F508CFTR and CFTR△PDZ diffusion in VX-809-treated and control MDCK cell lines. C, example trajectories for MDCK cells transiently expressing 3FLAG-tagged wtCFTR(black), △F508CFTR rescued by VX-809 (red), CFTR△PDZ ( blue ), and △F508CFTR△PDZ rescued by VX-809 (green). D, summary graph for CFTR dif- fusion in VX-809-treated MDCK transiently transfected with 3FLAG-tagged CFTR constructs. E, example trajectories from MDCK cell lines stably expressing wtCFTR ( left ) and △F508CFTR ( right ) treated with thapsigargin ( black)or CoPo-22 (red) or transiently expressing GFP-GRASP55 (blue ). F, graphs for △F508CFTR ( top ) and wtCFTR (bottom) diffusion in MDCK cell lines treated with thapsigargin or CoPo-22 or transiently expressing GFP-GRASP55. For reference, median and interquartile values for wtCFTR diffusive range are shown by dashed lines(bar 1, top ). G, GFP-GRASP55 is Golgi-localized by immunocytochemical analysis (scale bar, 10 μm). Scale bar in A (500 nm) refers to all trajectories. In panels Band D, statistically significant differences in populations are shown (*, p<0.001). SPT data for MDCK cell lines was derived from 140 –788 trajectories and for transiently transfected MDCK cells from 131–298 trajectories.
S7059	Tezacaftor (VX-661) Tezacaftor (VX-661) is a second F508del CFTR corrector and is believed to help CFTR protein reach the cell surface. Phase 2.	Sci Rep, 2018, 8(1):11404 J Cyst Fibros, 2018, 17(5):573-581 J Cyst Fibros, 2018, 10.1016/j.jcf.2018.07.001	(B)ΔISC response to forskolin observed in VX-661-treated CF HBE cells (P = 0.0021, VX661 vs. vehicle) was prevented by chronic VX-770 treatment and significantly different from VX-661-treated cells (#P=0.0126, VX661 vs. VX661+VX770). (C) The response to CFTR inh-172 that was observed in VX-661-treated cells (P = 0.0111, VX661 vs. vehicle) was significantly decreased in VX809+VX770 treated cells (#P = 0.0038, VX661 vs. VX661+VX770). Primary CF HBE cultures (ΔF508/ΔF508) were derived from 3 different patients, and 3-4 replicates were performed per patient.
S8795^新	FDL169 FDL169 is a CFTR corrector that is designed to fix and restore the function of the defective CFTR protein.

製品コード	製品説明	文献中Selleckの製品使用例	お客様のフィードバック
S6003	Ataluren (PTC124) Ataluren (PTC124) selectively induces ribosomal read-through of premature but not normal termination codons, with EC50 of 0.1 μM in HEK293 cells, may provide treatment for genetic disorders caused by nonsense mutations (e.g. CF caused by CFTR nonsense mutation). Phase 3.	Haematologica, 2019, 10.3324/haematol.2018.212118 J Cancer, 2019, 10(2):287-292 Am J Hematol, 2018, 93(4):527-536	Effect of systemic ataluren treatment on mice with the Pax6^Sey+/- phenotype. The black arrowhead indicates the lenticular stalk; the black arrow indicates the cornea; and the asterisk indicates the ciliary margin. WT, Pax6^+/+; Mt, Pax6^Sey/+; L, lens; r, retina. Original magnification, x5.
S7139	CFTRinh-172 CFTRinh-172 is a voltage-independent, selective CFTR inhibitor with K_i of 300 nM, showing no effects on MDR1, ATP-sensitive K+ channels, or a series of other transporters.	Nature, 2019, 567(7748):405-408 Int J Mol Med, 2018, 41(6):3501-3508 Reproduction, 2018, 156(3):261-268	CFTR inhibitor CFTR_inh172 leads to PGCs disorder in early zebrafish embryo. Analysis of localization of nanos1/vasa positive cells in embryos by WISH at 50% Epiboly stage with top view.
S7329	IOWH032 IOWH032 is a synthetic CFTR inhibitor with IC50 of 1.01 μM in CHO-CFTR cell based assays. Phase 2.
S8094	GlyH-101 GlyH-101 is a selective and reversible CFTR inhibitor with K_i of 4.3 μM.

製品コード	製品説明	文献中Selleckの製品使用例	お客様のフィードバック
S1144	Ivacaftor (VX-770) Ivacaftor (VX-770) is a selective potentiator of CFTR targeting G551D-CFTR and F508del-CFTR with EC50 of 100 nM and 25 nM in fisher rat thyroid cells, respectively.	EMBO J, 2019, 38(4) Cell Rep, 2019, 26(7):1701-1708 J Control Release, 2019, 296:225-231	(A) CFTR Western blot of normal (NL) and CF HBE cultures treated with VX-809 (5 uM) ± VX-770 (5 uM) for 48 hours. “*” indicates the mature, complex glycosylated form of CFTR, band C; “•” indicates the immature band B. (B) Turnover of rescued ΔF508 in BHK-21 cells. ΔF508 was rescued at 27°C in the presence of VX-809 ± VX-770 for 24 hours. After adding cycloheximide (200 ug/ml, 37°C), cells were lysed at the indicated times and analyzed by Western blotting.
S8698	GLPG1837 GLPG1837 is a novel CFTR potentiator with an EC50 value of 3 nM for F508del, showing enhanced efficacy on CFTR mutants harboring class III mutations compared to Ivacaftor.

製品コード

製品説明

文献中Selleckの製品使用例

お客様のフィードバック

S1144

Ivacaftor (VX-770)

Ivacaftor (VX-770) is a selective potentiator of CFTR targeting G551D-CFTR and F508del-CFTR with EC50 of 100 nM and 25 nM in fisher rat thyroid cells, respectively.

EMBO J, 2019, 38(4)

Cell Rep, 2019, 26(7):1701-1708

J Control Release, 2019, 296:225-231

S8698

GLPG1837

GLPG1837 is a novel CFTR potentiator with an EC50 value of 3 nM for F508del, showing enhanced efficacy on CFTR mutants harboring class III mutations compared to Ivacaftor.

製品コード	製品説明	文献中Selleckの製品使用例	お客様のフィードバック
S1565	VX-809 (Lumacaftor) VX-809 (Lumacaftor) acts to correct CFTR mutations common in cystic fibrosis by increasing mutant CFTR (F508del-CFTR) maturation,EC50 of 0.1 μM in fisher rat thyroid cells. Phase 3.	Am J Respir Crit Care Med, 2019, 199(1):123-126 EMBO J, 2019, 38(4) Cell Rep, 2019, 26(7):1701-1708	△F508CFTR diffusion is increased by different correction mechanisms. A, example trajectories from MDCK cell lines for wtCFTR (black) and △F508CFTR rescued by VX-809 treatment in the absence (red) and presence (blue ) of mCh-CFTR-C. B, summary graph for wtCFTR, r△F508CFTR and CFTR△PDZ diffusion in VX-809-treated and control MDCK cell lines. C, example trajectories for MDCK cells transiently expressing 3FLAG-tagged wtCFTR(black), △F508CFTR rescued by VX-809 (red), CFTR△PDZ ( blue ), and △F508CFTR△PDZ rescued by VX-809 (green). D, summary graph for CFTR dif- fusion in VX-809-treated MDCK transiently transfected with 3FLAG-tagged CFTR constructs. E, example trajectories from MDCK cell lines stably expressing wtCFTR ( left ) and △F508CFTR ( right ) treated with thapsigargin ( black)or CoPo-22 (red) or transiently expressing GFP-GRASP55 (blue ). F, graphs for △F508CFTR ( top ) and wtCFTR (bottom) diffusion in MDCK cell lines treated with thapsigargin or CoPo-22 or transiently expressing GFP-GRASP55. For reference, median and interquartile values for wtCFTR diffusive range are shown by dashed lines(bar 1, top ). G, GFP-GRASP55 is Golgi-localized by immunocytochemical analysis (scale bar, 10 μm). Scale bar in A (500 nm) refers to all trajectories. In panels Band D, statistically significant differences in populations are shown (*, p<0.001). SPT data for MDCK cell lines was derived from 140 –788 trajectories and for transiently transfected MDCK cells from 131–298 trajectories.
S7059	Tezacaftor (VX-661) Tezacaftor (VX-661) is a second F508del CFTR corrector and is believed to help CFTR protein reach the cell surface. Phase 2.	Sci Rep, 2018, 8(1):11404 J Cyst Fibros, 2018, 17(5):573-581 J Cyst Fibros, 2018, 10.1016/j.jcf.2018.07.001	(B)ΔISC response to forskolin observed in VX-661-treated CF HBE cells (P = 0.0021, VX661 vs. vehicle) was prevented by chronic VX-770 treatment and significantly different from VX-661-treated cells (#P=0.0126, VX661 vs. VX661+VX770). (C) The response to CFTR inh-172 that was observed in VX-661-treated cells (P = 0.0111, VX661 vs. vehicle) was significantly decreased in VX809+VX770 treated cells (#P = 0.0038, VX661 vs. VX661+VX770). Primary CF HBE cultures (ΔF508/ΔF508) were derived from 3 different patients, and 3-4 replicates were performed per patient.
S8795^新	FDL169 FDL169 is a CFTR corrector that is designed to fix and restore the function of the defective CFTR protein.

製品コード

製品説明

文献中Selleckの製品使用例

お客様のフィードバック

S1565

VX-809 (Lumacaftor)

VX-809 (Lumacaftor) acts to correct CFTR mutations common in cystic fibrosis by increasing mutant CFTR (F508del-CFTR) maturation,EC50 of 0.1 μM in fisher rat thyroid cells. Phase 3.

Am J Respir Crit Care Med, 2019, 199(1):123-126

EMBO J, 2019, 38(4)

Cell Rep, 2019, 26(7):1701-1708