RU2009142030A - ATF-BINDING CASSETTE MODULATORS - Google Patents

Abstract

 1. The compound of formula (I) !! or its pharmaceutically acceptable salt, where! Ar1 means a 5-6 membered aromatic monocyclic ring having 0-4 heteroatoms independently selected from nitrogen, oxygen or sulfur atoms, wherein the above ring is optionally fused to a 5-12 membered monocyclic or bicyclic, aromatic, partially unsaturated or saturated ring, where each ring contains 0-4 heteroatoms independently selected from nitrogen, oxygen or sulfur atoms, where Ar1 has m substituents, each independently selected from -WRW; ! W means a bond or an optionally substituted (C1-C6) alkylidene chain, where up to two methylene groups of the W residue are optionally and independently replaced by -CO-, -CS-, -COCO-, -CONR'-, -CONR'NR ', -CO2-, -OCO-, -NR'CO2-, -O-, -NR'CONR'-, -OCONR'-, -NR'NR'-, -NR'NR'CO-, -NR'CO- , -S-, -SO-, -SO2-, -NR'-, -SO2NR'-, -NR'SO2-, -NR'SO2NR'-; ! RW independently means R ', a halogen atom, NO2, CN, CF3 or OCF3; ! m is 0-5; ! each of R1, R2, R3, R4 and R5 independently means —X — RX; ! X means a bond or an optionally substituted (C1-C6) alkylidene chain, where up to two methylene groups of the X residue are optionally and independently replaced by -CO-, -CS-, -COCO-, -CONR'-, -CONR'NR ', -CO2-, -OCO-, -NR'CO2-, -O-, -NR'CONR'-, -OCONR'-, -NR'NR'-, -NR'NR'CO-, -NR'CO- , -S-, -SO-, -SO2-, -NR'-, -SO2NR'-, -NR'SO2- or -NR'SO2NR'-; ! RX independently means R ', a halogen atom, NO2, CN, CF3 or OCF3; ! R6 is a hydrogen atom, CF3, -OR ', -SR' or an optionally substituted (C1-C8) aliphatic group; ! R7 is a hydrogen atom or a (C1-C6) aliphatic group optionally substituted with a —X — RX group; ! R ′ is independently selected from a hydrogen atom or an optionally substituted group selected from a (C1-C8) aliphatic group, a 3-8 membered saturated, partially unsaturated or completely unsaturated monocyclic

Claims (75)

1. The compound of formula (I)

or its pharmaceutically acceptable salt, where Ar ¹ means a 5-6 membered aromatic monocyclic ring having 0-4 heteroatoms independently selected from nitrogen, oxygen or sulfur atoms, where the above ring is optionally fused to a 5-12 membered monocyclic or bicyclic, aromatic, partially unsaturated or saturated ring where each ring contains 0-4 heteroatoms independently selected from nitrogen, oxygen or sulfur atoms, where Ar ¹ has m substituents, each independently selected from —WR ^W ; W means a bond or an optionally substituted (C ₁ -C ₆ ) alkylidene chain, where up to two methylene groups of the W residue are optionally and independently replaced by -CO-, -CS-, -COCO-, -CONR'-, -CONR'NR ', -CO ₂ -, -OCO-, -NR'CO ₂ -, -O-, -NR'CONR'-, -OCONR'-, -NR'NR'-, -NR'NR'CO-, - NR'CO-, -S-, -SO-, -SO ₂ -, -NR'-, -SO ₂ NR'-, -NR'SO ₂ -, -NR'SO ₂ NR'-; R ^W independently means R ', a halogen atom, NO ₂ , CN, CF ₃ or OCF ₃ ; m is 0-5; each of R ¹ , R ² , R ³ , R ⁴ and R ⁵ independently means —X — R ^X ; X is a bond or an optionally substituted (C ₁ -C ₆ ) alkylidene chain, where up to two methylene groups of the X residue are optionally and independently replaced by -CO-, -CS-, -COCO-, -CONR'-, -CONR'NR ', -CO ₂ -, -OCO-, -NR'CO ₂ -, -O-, -NR'CONR'-, -OCONR'-, -NR'NR'-, -NR'NR'CO-, - NR'CO-, -S-, -SO-, -SO ₂ -, -NR'-, -SO ₂ NR'-, -NR'SO ₂ - or -NR'SO ₂ NR'-; R ^X independently means R ', a halogen atom, NO ₂ , CN, CF ₃ or OCF ₃ ; R ⁶ means a hydrogen atom, CF ₃ , -OR ', -SR' or an optionally substituted (C ₁ -C ₈ ) aliphatic group; R ⁷ represents a hydrogen atom or a (C ₁ -C ₆ ) aliphatic group optionally substituted with a —X — R ^X group; R 'is independently selected from a hydrogen atom or an optionally substituted group selected from a (C ₁ -C ₈ ) aliphatic group, a 3-8 membered saturated, partially unsaturated, or fully unsaturated monocyclic ring having 0-3 heteroatoms independently selected from nitrogen atoms oxygen or sulfur, or an 8-12 membered saturated, partially unsaturated or completely unsaturated bicyclic ring system having 0-5 heteroatoms independently selected from nitrogen, oxygen or sulfur atoms; or two existing radicals R 'together with the atom (s) to which they are attached form an optionally substituted 3-12 membered saturated, partially unsaturated or completely unsaturated monocyclic or bicyclic ring having 0-4 heteroatoms independently selected from nitrogen, oxygen or sulfur atoms; provided that: i) when R ¹ , R ² , R ³ , R ⁴ , R ⁵ , R ⁶ and R ^{7 are} hydrogen, then Ar ^{1 is} not phenyl, 2-methoxyphenyl, 4-methoxyphenyl, 2-methylphenyl, 2,6-dichlorophenyl , 2,4-dichlorophenyl, 2-bromophenyl, 4-bromophenyl, 4-hydroxyphenyl, 2,4-dinitrophenyl, 3,5-dicarboxyphenyl, 2,4-dimethylphenyl, 2,6-dimethylphenyl, 2-ethylphenyl, 3-nitro -4-methylphenyl, 3-carboxyphenyl, 2-fluorophenyl, 3-fluorophenyl, 3-trifluoromethylphenyl, 3-ethoxyphenyl, 4-chlorophenyl, 3-methoxyphenyl, 4-dimethylaminophenyl, 3,4-dimethylphenyl, 2-ethylphenyl-phenyl-4-phenyl-phenyl-4-ethylene ; ii) when R ¹ , R ² , R ³ , R ⁵ , R ⁶ and R ^{7 are} hydrogen, R ⁴ is methoxy, then Ar ^{1 is} not 2-fluorophenyl or 3-fluorophenyl; iii) when R ¹ , R ³ , R ⁴ , R ⁵ , R ⁶ and R ^{7 are} hydrogen, R ² is 1,2,3,4-tetrahydroisoquinolin-1-ylsulfonyl, then Ar ^{1 is} not 3-trifluoromethylphenyl; iv) when R ¹ , R ² , R ³ , R ⁴ , R ⁵ and R ^{7 are} hydrogen, R ⁶ is methyl, then Ar ^{1 is} not phenyl; v) when R ¹ , R ⁴ , R ⁵ , R ⁶ and R ^{7 are} hydrogen, R ² and R ³ taken together are methylenedioxy, then Ar ^{1 is} not 4-chlorophenyl, 4-bromophenyl, 4-nitrophenyl, 4-carboethoxyphenyl 6-ethoxybenzothiazol-2-yl, 6-carboethoxybenzothiazol-2-yl, 6-halogenbenzothiazol-2-yl, 6-nitrobenzothiazol-2-yl or 6-thiocyanobenzothiazol-2-yl; vi) when R ¹ , R ⁴ , R ⁵ , R ⁶ and R ^{7 are} hydrogen, R ² and R ³ taken together are methylenedioxy, then Ar ^{1 is} not 4-substituted phenyl, where the above substituent is —SO ₂ NHR ^XX , R ^XX is 2-pyridinyl, 4-methyl-2-pyrimidinyl, 3,4-dimethyl-5-isoxazolyl; vii) when R ¹ , R ² , R ³ , R ⁴ , R ⁵ , R ⁶ and R ^{7 are} hydrogen, then Ar ^{1 is} not thiazol-2-yl, 1H-1,2,4-triazol-3-yl or 1H-1,3,4-triazol-2-yl; viii) when R ¹ , R ² , R ³ , R ⁵ , R ⁶ and R ^{7 are} hydrogen, R ⁴ is CF ₃ , OMe, a chlorine atom, SCF ₃ or OCF ₃ , then Ar ^{1 is} not 5-methyl-1 , 2-oxazol-3-yl, thiazol-2-yl, 4-fluorophenyl, pyrimidin-2-yl, 1-methyl-1,2- (1H) -pyrazol-5-yl, pyridin-2-yl, phenyl , N-methylimidazol-2-yl, imidazol-2-yl, 5-methylimidazol-2-yl, 1,3-oxazol-2-yl or 1,3,5- (1H) -triazol-2-yl; ix) when R ¹ , R ² , R ³ , R ⁴ , R ⁵ , R ⁶ and R ^{7 are} each a hydrogen atom, then Ar ^{1 is} not pyrimidin-2-yl, 4,6-dimethylpyrimidin-2-yl, 4 -methoxy-6-methyl-1,3,5-triazin-2-yl, 5-bromopyridin-2-yl, pyridin-2-yl or 3,5-dichloropyridin-2-yl; x) when R ¹ , R ² , R ³ , R ⁴ , R ⁵ and R ^{7 are} each a hydrogen atom, R ⁶ is a hydroxy group, then Ar ^{1 is} not 2,6-dichloro-4-aminosulfonylphenyl; xi) when R ² or R ³ is optionally substituted N-piperazyl, N-piperidyl or N-morpholinyl, then Ar ^{1 is} not an optionally substituted ring selected from thiazol-2-yl, pyridyl, phenyl, thiadiazolyl, benzothiazol-2- sludge or indazolyl; xii) when R ² is an optionally substituted cyclohexylamino group, then Ar ¹ is not optionally substituted phenyl, pyridyl or thiadiazolyl; xiii) Ar ¹ is not optionally substituted with tetrazolyl; xiv) when R ² , R ⁴ , R ⁵ , R ⁶ and R ^{7 are} each a hydrogen atom, R ¹ and R ^{3 are} both CF ₃ , a chlorine atom, methyl or methoxy group, then Ar ^{1 is} not 4,5-dihydro -1,3-thiazol-2-yl, tizol-2-yl or [3,5-bis (trifluoromethyl) -1H-pyrazol-1-yl] phenyl; xv) when R ¹ , R ⁴ , R ⁵ , R ⁶ and R ^{7 are} each a hydrogen atom, Ar ¹ is thiazol-2-yl, then neither R ² nor R ³ are isopropyl, a chlorine atom or CF ₃ ; xvi) when Ar ¹ is 4-methoxyphenyl, 4-trifluoromethylphenyl, 2-fluorophenyl, phenyl or 3-chlorophenyl, then: a) when R ¹ , R ² , R ⁴ , R ⁵ , R ⁶ and R ^{7 are} each a hydrogen atom, then R ^{3 is} not a methoxy group; or b) when R ¹ , R ³ , R ⁴ , R ⁵ , R ⁶ and R ^{7 are} each a hydrogen atom, then R ^{2 is} not a chlorine atom; or c) when R ¹ , R ² , R ³ , R ⁵ , R ⁶ and R ^{7 are} each a hydrogen atom, then R ^{4 is} not a methoxy group; or d) when R ¹ , R ³ , R ⁴ , R ⁶ and R ^{7 are} each a hydrogen atom, R ⁵ is ethyl, then R ^{2 is} not a chlorine atom; e) when R ¹ , R ² , R ⁴ , R ⁵ , R ⁶ and R ^{7 are} each a hydrogen atom, then R ^{3 is} not a chlorine atom; xvii) when R ¹ , R ³ , R ⁴ , R ⁵ , R ⁶ and R ^{7 are} each a hydrogen atom, R ² is CF ₃ or OCF ₃ , then Ar ^{1 is} not [3,5-bis (trifluoromethyl) -1H -pyrazol-1-yl] phenyl; xviii) when R ¹ , R ² , R ⁴ , R ⁵ , R ⁶ and R ^{7 are} each a hydrogen atom, R ³ is a hydrogen atom or CF ₃ , then Ar ^{1 is} not phenyl substituted with —OCH ₂ CH ₂ phenyl, - OCH ₂ CH ₂ - (2-trifluoromethylphenyl), -OCH ₂ CH ₂ - (6,7-dimethoxy-1,2,3,4-tetrahydroisoquinolin-2-yl) or substituted 1H-pyrazol-3-yl; xix) the following two connections are excluded

xx) the compound is not N- (5-hydroxy-2,4-di-tert-butylphenyl) -4-oxo-1H-quinoline-3-carboxamide; and xxi) the compound is not a pharmaceutically acceptable salt of N- (5-hydroxy-2,4-di-tert-butylphenyl) -4-oxo-1H-quinoline-3-carboxamide. 2. The compound according to claim 1, where Ar ^{1 is} selected from

where ring A ₁ means a 5-6 membered aromatic monocyclic ring having 0-4 heteroatoms independently selected from nitrogen, oxygen or sulfur atoms; or A ₁ and A ₂ taken together mean an 8-14 membered aromatic, bicyclic or tricyclic aryl ring, where each ring contains 0-4 heteroatoms independently selected from nitrogen, oxygen or sulfur atoms. 3. The compound according to claim 2, where A ₁ means an optionally substituted 6-membered aromatic ring having 0-4 heteroatoms, where the above heteroatom is a nitrogen atom. 4. The compound according to claim 2, where A ₁ means optionally substituted phenyl. 5. The compound according to claim 2, where A ₂ means an optionally substituted 6-membered aromatic ring having 0-4 heteroatoms independently selected from nitrogen, oxygen or sulfur atoms. 6. The compound according to claim 2, where A ₂ means an optionally substituted 5-membered aromatic ring having 0-3 heteroatoms independently selected from nitrogen, oxygen or sulfur atoms. 7. The compound according to claim 2, where A ₂ means a 5-membered aromatic ring having 1-2 nitrogen atoms. 8. The compound according to claim 2, where A _{2 is} selected from

where the ring A _{2 is} condensed with the ring A ₁ at two adjacent ring atoms. 9. The compound according to claim 1, where R ⁵ and R ⁶ mean hydrogen. 10. The compound according to claim 9, where R ³ and R ⁴ mean simultaneously hydrogen; R ¹ means a hydrogen atom or —OH; and R ² means a hydrogen atom or a fluorine atom. 11. The compound of claim 10, where R ⁷ means a hydrogen atom. 12. The compound according to claim 1, where each available WR ^W means independently (C ₁ -C ₃ ) alkyl, (C ₁ -C ₃ ) perhaloalkyl, -O- (C ₁ -C ₃ ) alkyl, -CF ₃ , - OCF ₃ , -SCF ₃ , -F, -Cl, -Br or -COOR ', -COR', -O (CH ₂ ) ₂ N (R ') (R'), -O (CH ₂ ) N (R ') (R'), -CON (R ') (R'), - (CH ₂ ) ₂ OR ', - (CH ₂ ) OR', optionally substituted monocyclic or bicyclic aromatic ring, optionally substituted arylsulfone, optionally substituted 5 a membered heteroaryl ring, —N (R ′) (R ′), - (CH ₂ ) ₂ N (R ′) (R ′) or - (CH ₂ ) N (R ′) (R ′). 13. The compound according to item 12, where each available WR ^{W is} selected from a halogen atom, a cyano group, CF ₃ , CHF ₂ , OCHF ₂ , Me, Et, CH (Me) ₂ , CHMeEt, n-propyl, tert-butyl, OMe, OEt, OPh, O-fluorophenyl, O-difluorophenyl, O-methoxyphenyl, O-tolyl, O-benzyl, SMe, SCF ₃ , SCHF ₂ , Set, CH ₂ CN, NH ₂ , NHMe, N (Me) ₂ , NHEt, N (Et) ₂ , C (O) CH ₃ , C (O) Ph, C (O) NH ₂ , SPh, SO ₂ - (aminopyridyl), SO ₂ NH ₂ , SO ₂ Ph, SO ₂ NHPh, SO ₂ -N-morpholino groups, SO ₂ -N-pyrrolidyl, N-pyrrolyl, N-morpholino groups, 1-piperidyl, phenyl, benzyl, (cyclohexylmethylamino) methyl, 4-methyl-2,4-dihydropyrazol-3-one-2-yl, benzimidazol-2-yl, furan-2-yl, 4-methyl-4H- [1,2,4] triazol-3-yl, 3- (4'-chlorophenyl) - [1,2,4] oxadiazol-5-yl, NHC (O) Me, NHC (O) Et, NHC (O) Ph, NHSO ₂ Me, 2-indolyl, 5-indolyl, -CH ₂ CH ₂ OH, -OCF ₃ , O- (2,3-dimethylphenyl), 5-methylfuryl, -SO ₂ -N-piperidyl, 2-tolyl, 3-tolyl, 4-tolyl, O-butyl, NHCO ₂ C (Me) ₃ , CO ₂ C (Me) ₃ , isopropenyl, n-butyl, O- (2,4-dichlorophenyl), NHSO ₂ PhMe, O- (3-chloro-5-trifluoromethyl-2-pyridyl), phenylhydroxymethyl, 2,5-dimethylpyrrolyl, NHCOCH ₂ C (Me) ₃ , O- (2-tert-butyl) phenyl, 2,3-dimethylphenyl, 3,4-dimethylphenyl, 4-hydroxymethylphenyl, 4-dimethylaminophenyl, 2-trifluoromethylphenyl, 3-trifluoromethylphenyl, 4-trifluoromethyl-phenyl, 4-cyanomethylphenyl, 4-isobutylphenyl, 3-pyridyl, 4-pyridyl, 4-isopropylphenyl, 3-isopropylphenyl, 2-methoxyphenyl, 3-methoxyphenyl, 4-methoxyphenyl, 3,4-methylenedioxyphenyl, 2-ethoxyphenyl, 3-ethoxyphenyl, 4-ethoxyphenyl, 2-methylthiophenyl, 4-methylthiophenyl, 2,4-dimethoxyphenyl, 2,5-dimethoxyphenyl, 2,6-dimethoxyphenyl, 3,4-dimethoxyphenyl, 5-chloro-2-methoxyphenyl, 2-OCF ₃ -phenyl, 3-trifluoromethoxyphenyl, 4-trifluoromethoxyphenyl, 2-phenoxyphenyl, 4-phenoxyphenyl, 2-fluoro-3-methoxyphenyl, 2,4-dimethoxy-5-pyrimidyl, 5-isopropyl-2-methoxyphenyl, 2-fluorophenyl, 3-fluorophenyl, 4-fluorophenyl, 3-cyanophenyl, 3-chlorophenyl, 4-chlorophenyl, 2,3-difluorophenyl, 2,4-difluorophenyl, 2,5-difluorophenyl, 3,4-difluorophenyl, 3,5-difluorophenyl, 3-chloro-4-fluorophenyl, 3,5-dichlorophenyl, 2,5-dichlorophenyl, 2,3-dichlorophenyl, 3,4-dichlorophenyl, 2,4-dichlorophenyl, 3-methoxycarbonylphenyl, 4-methoxycarbonylphenyl, 3-isopropyloxycarbonylphenyl, 3-acetamidophenyl, 4-fluoro-3-methylphenyl, 4-methanesulfinyl phenyl, 4-methanesulfonylphenyl, 4-N- (2-N, N-dimethylaminoethyl) carbamoylphenyl, 5-acetyl-2-thienyl, 2-benzothienyl, 3-benzothienyl, furan-3-yl, 4-methyl-2-thienyl, 5-cyano-2-thienyl, N'-phenylcarbonyl-N-piperazinyl, -NHCO ₂ Et, -NHCO ₂ Me, N-pyrrolidinyl, -NHSO ₂ (CH ₂ ) ₂ -N-piperidine, -NHSO ₂ (CH ₂ ) ₂ -N-morpholine, -NHSO ₂ (CH ₂ ) ₂ N (Me) ₂ , -COCH ₂ N (Me) COCH ₂ NHMe, —CO ₂ Et, O-propyl, —CH ₂ CH ₂ NHCO ₂ C (Me) ₃ , hydroxyl, aminomethyl, pentyl, adamantyl, cyclopentyl, ethoxyethyl, C (Me) ₂ CH ₂ OH, -C (Me) ₂ CO ₂ Et, -CHOHMe, CH ₂ CO ₂ Et, -C (Me) ₂ CH ₂ NHCO ₂ C (Me) ₃ , O (CH ₂ ) ₂ OЕt, O (CH ₂ ) ₂ OH, CO ₂ Me, hydroxymethyl, 1-methyl-1-cyclohexyl, 1-methyl-1-cyclooctyl, 1-methyl-1-cycloheptyl, C (Et) ₂ C (Me) ₃ , C (Et) ₃ , CONHCH ₂ CH (Me) ₂ , 2-aminomethylphenyl, ethenyl, 1-piperidinylcarbonyl, ethynyl, cyclohexyl, 4-methylpiperidinyl, -CO ₂ Me, -C (Me) ₂ CH ₂ NHCO ₂ CH ₂ CH (Me) ₂ , -C (Me) ₂ CH ₂ NHCO ₂ CH ₂ CH ₂ CH ₃ , -C (Me) ₂ CH ₂ NHCO ₂ Et, -C (Me) ₂ CH ₂ NHCO ₂ Me, -C (Me) ₂ CH ₂ NHCO ₂ CH ₂ C (Me) ₃ , -CH ₂ NHCOCF ₃ , -CH ₂ NHCO ₂ C (Me) ₃ , -C (Me) ₂ CH ₂ NHCO ₂ (CH ₂ ) ₃ CH ₃ , -C (Me) ₂ CH ₂ NHCO ₂ (CH ₂ ) ₂ OMe, C (OH) (CF ₃ ) ₂ , -C (Me) ₂ CH ₂ NHCO ₂ CH ₂ -tetrahydrofuran-3-yl, C (Me) ₂ CH ₂ O (CH ₂ ) ₂ OMe or 3-ethyl-2,6-dioxopiperidin-3-yl. 14. The compound according to claim 2, where the above compound corresponds to formula IIA or formula IIB

15. The compound according to claim 2, where the above compound corresponds to formula IIIA , formula IIIB , formula IIIC , formula IIID or formula IIIE

Where each of X ₁ , X ₂ , X ₃ , X _4, and X ₅ is independently selected from CH or N; and X ₆ means O, S or NR '. 16. The compound according to clause 15, where X ₁ X ₂ X ₃ X ₄ and X ₅ taken together with WR ^W and m mean optionally substituted phenyl. 17. The compound of clause 15, where X ₁ , X ₂ , X ₃ , X ₄ and X ₅ taken together in the compound of formula IIIA mean an optionally substituted ring selected from

18. The compound according to clause 15, where X ₁ , X ₂ , X ₃ , X ₄ , X ₅ or X ₆ taken together with the ring A ₂ in the compounds of formula IIIB , formula IIIC , formula IIID or formula IIIE mean an optionally substituted ring, selected from

19. The compound according to clause 15, where the above compound corresponds to formula IVA , formula IVB or formula IVC

20. The compound according to claim 19, where the ring A ₂ means an optionally substituted, saturated, unsaturated or aromatic 5-7-membered ring having 0-3 heteroatoms selected from O, S or N. 21. The compound according to claim 19, where the above compound corresponds to the formula VA-1

where each of WR ^W2 and WR ^W4 is independently selected from a hydrogen atom, CN, CF ₃ , a halogen atom, a linear or branched (C ₁ -C ₆ ) alkyl group, a 3-12 membered cycloaliphatic group, phenyl, (C ₅ -C ₁₀ ) a heteroaryl or (C ₃ -C ₇ ) heterocyclic group, wherein the aforementioned heteroaryl or heterocyclic group includes up to 3 heteroatoms selected from O, S or N atoms, where WR ^W2 and WR ^{W4 are} independently and optionally substituted with up to three substituents selected from -OR ', -CF ₃ , -OCF ₃ , SR', S (O) R ', SO ₂ R', -SCF ₃ , halogen atom, CN, -COOR ', -COR', O (CH ₂ ) ₂ N (R ') (R'), -O (CH ₂ ) N (R ') (R'), -CON (R ') (R'), - (CH ₂ ) ₂ OR ', - (CH ₂ ) OR ', -CH ₂ N, optionally substituted phenyl or phenoxy groups, N (R') (R '), -NR'C (O) OR', -NR'C (O) R ', - (CH ₂ ) ₂ N (R ') (R') or - (CH ₂ ) N (R ') (R'); and WR ^{W5 is} selected from a hydrogen atom, —OH, NH ₂ , CN, CHF ₂ , NHR ′, N (R ′) ₂ , —NHC (O) R ′, —NHC (O) OR ′, —NHSO ₂ R ′, -OR ', CH ₂ OH, CH ₂ N (R') ₂ , C (O) OR ', SO ₂ NHR', SO ₂ N (R ') ₂ or -CH ₂ NHC (O) OR'; or WR ^W4 and WR ^W5 taken together form a 5-7 membered ring having 0-3 heteroatoms selected from N, O or S atoms, where the above ring is optionally substituted with up to three WR ^W substituents. 22. The compound according to item 21, where each of WR ^W2 and WR ^{W4 is} independently selected from a hydrogen atom, CN, CF ₃ , a halogen atom, a linear or branched (C ₁ -C ₆ ) alkyl group, a 3-12 membered cycloaliphatic group, or phenyl, where the above WR ^W2 and WR ^W4 independently and optionally substituted with up to three substituents selected from —OR ′, —CF ₃ , —OCF ₃ , —SCF ₃ , halogen atom, —COOR ′, —COR ′, —O (CH ₂ ) ₂ N (R ′) (R '), -O (CH ₂ ) N (R') (R '), -CON (R') (R '), - (CH ₂ ) ₂ OR', - (CH ₂ ) OR ', optional substituted phenyl, N (R ') (R'), -NC (O) OR ', -NC (O) R', - (CH ₂ ) ₂ N (R ') (R') or - (CH ₂ ) N (R ') (R'); and WR ^{W5 is} selected from a hydrogen atom, —OH, NH ₂ , CN, CHF ₂ , NHR ′, N (R ′) ₂ , —NHC (O) R ′, —NHC (O) OR ′, —NHSO ₂ R ′, -OR ', CH ₂ OH, C (O) OR', SO ₂ NHR 'or -CH ₂ NHC (O) O- (R'). 23. The compound according to item 22, where WR ^W2 means a phenyl ring optionally substituted with up to three substituents selected from —OR ′, —CF ₃ , —OCF ₃ , SR ′, S (O) R ′, SO ₂ R ′, —SCF ₃ , halogen atom, CN , -COOR ', -COR', -O (CH ₂ ) ₂ N (R ') (R'), -O (CH ₂ ) N (R ') (R'), -CON (R ') (R '), - (CH ₂ ) ₂ OR', -CH ₂ ) OR ', -CH ₂ N, optionally substituted phenyl or phenoxy groups, N (R') (R '), -NR'C (O) OR', -NR'C (O) R ', -CH ₂ ) ₂ N (R') (R ') or - (CH ₂ ) N (R') (R '); WR ^W4 means linear or branched (C ₁ -C ₆ ) alkyl; and WR ^W5 means OH. 24. The compound of claim 22, wherein each of WR ^W2 and WR ^{W4 is} independently selected from CF ₃ , a halogen atom, CN, or linear or branched (C ₁ -C ₆ ) alkyl. 25. The compound of claim 24, wherein each of WR ^W2 and WR ^{W4 is} independently selected from optionally substituted n-propyl, isopropyl, n-butyl, sec-butyl, tert-butyl, 1,1-dimethyl-2-hydroxyethyl, 1 , 1-dimethyl-2- (ethoxycarbonyl) ethyl, 1,1-dimethyl-3- (tert-butoxycarbonylamino) propyl or n-pentyl. 26. The compound according A.25, where each of WR ^W2 and WR ^W4 means a linear or branched (C ₁ -C ₆ ) alkyl. 27. The compound according to item 21, where WR ^W5 selected from a hydrogen atom, CHF ₂ , NH ₂ , CN, NHR ', N (R') ₂ , CH ₂ N (R ') ₂ , -NHC (O) R' , -NHC (O) OR ', -OR', -C (O) OR 'or SO ₂ NHR'. 28. The compound according to item 21, where WR ^W5 selected from a hydrogen atom, NH ₂ , CN, CHF ₂ , NH (C ₁ -C ₆ ) alkyl, N ((C ₁ -C ₆ ) alkyl) ₂ , NHC (O) (C ₁ -C ₆ ) alkyl, -CH ₂ NCH (O) O (C ₁ -C ₆ ) alkyl, -NHC (O) O (C ₁ -C ₆ ) alkyl, -OH, -O (C ₁ -C ₆ ) alkyl, -C (O) O (C ₁ -C ₆ ) alkyl, -CH ₂ O (C ₁ -C ₆ ) alkyl or SO ₂ NH ₂ . 29. The compound of claim 28, wherein WR ^{W5 is} selected from —OH, OMe, NH ₂ , —NHMe, —N (Me) ₂ , —CH ₂ NH ₂ , CH ₂ OH, NHC (O) OMe, NHC (O ) OEt, CN, CHF ₂ , -CH ₂ NHC (O) O (tert-butyl), -O-ethoxyethyl, -O-hydroxyethyl, -C (O) OMe or -SO ₂ NH ₂ . 30. The compound according to item 21, where the above compound has one, preferably several, more preferably all of the following features: but. WR ^W2 is a hydrogen atom; b. WR ^W4 means a linear or branched (C ₁ -C ₆ ) alkyl or a monocyclic or bicyclic aliphatic group; and from. WR ^{W5 is} selected from a hydrogen atom, CN, CHF ₂ , NH ₂ , NH (C ₁ -C ₆ ) alkyl, N ((C ₁ -C ₆ ) alkyl) ₂ , NHC (O) (C ₁ -C ₆ ) alkyl, -NHC (O) O (C ₁ -C ₆ ) alkyl, -CH ₂ C (O) O (C ₁ -C ₆ ) alkyl, -OH, -O (C ₁ -C ₆ ) alkyl; -C (O) O (C ₁ -C ₆ ) alkyl or SO ₂ NH ₂ . 31. The compound according to item 21, where the above compound has one, preferably several, more preferably all of the following features: but. WR ^W2 is a halogen atom, (C ₁ -C ₆ ) alkyl, CF ₃ , CN or phenyl optionally substituted with up to three substituents selected from (C ₁ -C ₄ ) alkyl, -O (C ₁ -C ₄ ) alkyl or a halogen atom; b. WR ^W4 is CF ₃ , a halogen atom, a (C ₁ -C ₆ ) alkyl or (C ₆ -C ₁₀ ) cycloaliphatic group; and from. WR ^W5 means OH, NH ₂ , NH (C ₁ -C ₆ ) alkyl or N (C ₁ -C ₆ ) alkyl. 32. The compound according to claim 19, where the above compound corresponds to the formula VA-2

Where Y is CH ₂ , C (O) O, C (O) or S (O) ₂ ; and m is 0-4. 33. The compound according to claim 19, where the above compound corresponds to the formula VA-3

Where Q is W; R ^Q means R ^W ; m is 0-4; and n is 0-4. 34. The compound according to claim 19, where the above compound corresponds to the formula VA-4

35. The compound according to claim 19, where the above compound corresponds to the formula VA-5

where m is 0-4. 36. The compound according to claim 19, where the above compound corresponds to the formula VA-6

Where ring B means a 5-7 membered monocyclic or bicyclic, heterocyclic or heteroaryl ring optionally substituted with up to n residues —QR ^Q ; Q is W; R ^Q means R ^W ; m is 0-4; and n is 0-4. 37. The compound of claim 36, wherein ring B is a 5-7 membered monocyclic, heterocyclic ring containing up to 2 heteroatoms selected from O, S or N atoms, optionally substituted with up to n residues -QR ^Q. 38. The compound of claim 36, wherein ring B is a 5-6 membered monocyclic heteroaryl ring containing up to 2 heteroatoms selected from O, S, or N atoms, optionally substituted with up to n residues —QR ^Q. 39. The compound of claim 36, wherein ring B is selected from N-morpholinyl, N-piperidinyl, 4-benzoylpiperazin-1-yl, pyrrolidin-1-yl or 4-methylpiperidin-1-yl, benzimidazol-2-yl, 5 -methylfuran-2-yl, 2,5-dimethylpyrrol-1-yl, pyridin-4-yl, indol-5-yl, indol-2-yl, 2,4-dimethoxypyrimidin-5-yl, furan-2-yl furan-3-yl, 2-acylthien-2-yl, benzothiophen-2-yl, 4-methylthien-2-yl, 5-cyanothien-2-yl, 3-chloro-5-trifluoromethylpyridin-2-yl. 40. The compound according to claim 19, where the above compound corresponds to the formula VB-1

Where one of Q ₁ and Q ₃ is N (WR ^W ), and the other of Q ₁ and Q _{3 is} selected from O, S or N (WR ^W ); Q ₂ means C (O), CH ₂ —C (O), C (O) —CH ₂ , CH ₂ , CH ₂ —CH ₂ , CF ₂ or CF ₂ —CF ₂ ; and m is 0-3. 41. The compound of claim 40, wherein Q ₃ is N (WR ^W ), where WR ^W is a hydrogen atom, a (C ₁ -C ₆ ) aliphatic group, C (O) (C ₁ -C ₆ ) an aliphatic group, or C (O) O- (C ₁ -C ₆ ) aliphatic group. 42. The compound according to paragraph 41, where Q ₂ means C (O), CH ₂ , CH ₂ -CH ₂ , Q ₁ means O. 43. The compound according to claim 19, where the above compound corresponds to the formula VB-2

Where R ^W1 is a hydrogen atom or a (C ₁ -C ₆ ) aliphatic group; each of R ^W3 is a hydrogen atom or a (C ₁ -C ₆ ) aliphatic group; or both R ^W3 taken together form a (C ₃ -C ₆ ) cycloalkyl or heterocyclic ring containing up to 2 heteroatoms selected from O, S or NR 'atoms, where the above ring is optionally substituted with up to two WR ^W substituents; and m is 0-4. 44. The compound according to item 43, where WR ^W1 means a hydrogen atom, (C ₁ -C ₆ ) aliphatic group, C (O) (C ₁ -C ₆ ) aliphatic group or C (O) O- (C ₁ -C ₆ ) an aliphatic group. 45. The compound according to item 43, where each R ^W3 means a hydrogen atom, (C ₁ -C ₄ ) alkyl; or both R ^W3 taken together form a (C ₃ -C ₆ ) cycloaliphatic ring or a 5-7 membered heterocyclic ring containing up to two heteroatoms selected from O, S or N atoms, where the cycloaliphatic or heterocyclic ring is optionally substituted with up to three substituents selected from WR ^W1 . 46. The compound according to claim 19, where the above compound corresponds to the formula VB-3

Where Q ₄ means a bond, C (O), C (O) O or S (O) ₂ ; R ^W1 is a hydrogen atom or a (C ₁ -C ₆ ) aliphatic group; and m is 0-4. 47. The compound according to claim 19, where the above compound corresponds to the formula VB-4

where m is 0-4. 48. The compound according to claim 19, where the above compound corresponds to the formula VB-5

Where A ₂ ring means phenyl or a 5-6 membered heteroaryl ring, where A ₂ ring and the phenyl ring fused to it together contain up to 4 substituents independently selected from WR ^W ; and m is 0-4. 49. The compound of claim 48, wherein ring A _{2 is} selected from

where the above ring is optionally substituted. 50. The compound according to claim 19, where the above compound corresponds to the formula VB-5-a

Where G ₄ means a hydrogen atom, a halogen atom, CN, CF ₃ , CHF ₂ , CH ₂ F, an optionally substituted (C ₁ -C ₆ ) aliphatic group, an aryl- (C ₁ -C ₆ ) alkyl or phenyl, where G _{4 is} optional substituted by up to 4 substituents WR ^W , where up to two methylene groups of the above (C ₁ -C ₆ ) aliphatic group or (C ₁ -C ₆ ) alkyl are optionally replaced by -CO-, -CONR'-, -CO ₂ -, -OCO-, -NR'CO ₂ -, -O-, -NR'CONR'-, -OCONR'-, -NR'CO-, -S-, -NR'-, -SO ₂ NR'-, - NR'SO ₂ - or -NR'SO ₂ NR'-; G ₅ means a hydrogen atom or an optionally substituted (C ₁ -C ₆ ) aliphatic group; where the above indole ring system is additionally optionally substituted with up to three substituents independently selected from WR ^W. 51. The compound of claim 50, wherein G ₄ is a hydrogen atom and G ₅ is a (C ₁ -C ₆ ) aliphatic group, wherein the above aliphatic group is optionally substituted with (C ₁ -C ₆ ) alkyl, a halogen atom, a cyano group, or CF ₃ , and where up to two methylene groups of the above (C ₁ -C ₆ ) aliphatic group or (C ₁ -C ₆ ) alkyl are optionally replaced by -CO-, -CONR'-, -CO ₂ -, -OCO-, - NR'CO ₂ -, -O-, -NR'CONR'-, -OCONR'-, -NR'CO-, -S-, -NR'-, -SO ₂ NR'-, -NR'SO ₂ - or -NR'SO ₂ NR'-. 52. The compound of claim 50, wherein G ₄ is a hydrogen atom and G ₅ is a cyano group, methyl, ethyl, propyl, isopropyl, butyl, sec-butyl, tert-butyl, cyanomethyl, methoxyethyl, CH ₂ C (O) OMe , (CH ₂ ) ₂ —NHC (O) O-tert-butyl or cyclopentyl. 53. The compound of claim 50, wherein G ₅ is a hydrogen atom and G ₄ is a halogen atom, a (C ₁ -C ₆ ) aliphatic group or phenyl, wherein the above aliphatic group or phenyl is optionally substituted (substituted) (C ₁ -C ₆ ) alkyl, a halogen atom, a cyano group or CF ₃ , and where up to two methylene groups of the above (C ₁ -C ₆ ) aliphatic group or (C ₁ -C ₆ ) alkyl are optionally replaced by -CO-, -CONR'-, -CO ₂ -, -OCO-, -NR'CO ₂ -, -O-, -NR'CONR'-, -OCONR'-, -NR'CO-, -S-, -NR'-, -SO ₂ NR'-, -NR'SO ₂ - or -NR'SO ₂ NR'-. 54. The compound of claim 50, wherein G ₅ is a hydrogen atom and G ₄ is a halogen atom, ethoxycarbonyl, tert-butyl, 2-methoxyphenyl, 2-ethoxyphenyl, (4-C (O) NH (CH ₂ ) ₂ - NMe ₂ ) phenyl, 2-methoxy-4-chlorophenyl, pyridin-3-yl, 4-isopropylphenyl, 2,6-dimethoxyphenyl, sec-butylaminocarbonyl, ethyl, tert-butyl or piperidin-1-ylcarbonyl. 55. The compound according to claim 1, where the above compound is selected from table 1. 56. The compound corresponding to the formula AI

or its salt, Where G ₁ means a hydrogen atom, R ', C (O) R', C (S) R ', S (O) ₂ R', Si (CH ₃ ) ₂ R ', P (O) (OR') ₃ , P (S) (OR ') ₃ or B (OR') ₂ ; G ₂ means a halogen atom, CN, CF ₃ , isopropyl or phenyl, wherein the above isopropyl or phenyl is optionally substituted with up to 3 substituents independently selected from WR ^W , where W and R ^W are as defined above for Formula I and its embodiments; G ₃ means isopropyl or a (C ₃ -C ₁₀ ) cycloaliphatic ring, wherein the aforementioned G _{3 is} optionally substituted with up to three substituents independently selected from WR ^W , where W and R ^W are as defined above for formula I and its embodiments; provided that when G ₁ is methoxy, G ₃ is tert-butyl, then G _{2 is} not 2-amino-4-methoxy-5-tert-butylphenyl. 57. The compound of claim 56, wherein G ₁ means a hydrogen atom; G ₂ means a halogen atom or isopropyl, where the above isopropyl is optionally substituted with up to three substituents independently selected from R '; and G ₃ means isopropyl or a (C ₃ -C ₁₀ ) cycloaliphatic ring, wherein the above G _{3 is} optionally substituted with up to three substituents independently selected from R ′. 58. The compound according to clause 57, where G ₁ means a hydrogen atom; G ₂ means a halogen atom, preferably a fluorine atom; and G ₃ means a (C ₃ -C ₁₀ ) cycloaliphatic ring, wherein the above G _{3 is} optionally substituted with up to three substituents independently selected from methyl, ethyl, propyl or butyl. 59. The compound of claim 56, wherein G ₁ means a hydrogen atom; G ₂ means CN, a halogen atom or CF ₃ ; and G ₃ means isopropyl or a (C ₃ -C ₁₀ ) cycloaliphatic ring, wherein the above G _{3 is} optionally substituted with up to three substituents independently selected from R ′. 60. The compound of claim 56, wherein G ₁ means a hydrogen atom; G ₂ is phenyl optionally substituted with up to three substituents independently selected from —O— (C ₁ -C ₄ ) alkyl, CF ₃ , a halogen atom or CN; and G ₃ means isopropyl or a (C ₃ -C ₁₀ ) cycloaliphatic ring, wherein the above G _{3 is} optionally substituted with up to three substituents independently selected from R ′. 61. The compound of claim 56, wherein G _{3 is} selected from optionally substituted cyclopentyl, cyclohexyl, cycloheptyl or adamantyl. 62. The compound of claim 56, wherein G ₃ is a branched (C ₃ -C ₈ ) aliphatic chain. 63. The compound according to the formula A-II

or its salt, where G ₄ represents a hydrogen atom, a halogen atom, CN, CF _3, CHF _2, CH ₂ F, optionally substituted (C ₁ -C ₆₎ aliphatic, aralkyl, or phenyl ring optionally substituted with up to four substituents WR ^W; G ₅ means a hydrogen atom or an optionally substituted (C ₁ -C ₆ ) aliphatic group; provided that both G ₄ and G _{5 are} simultaneously not hydrogen; where the above indole ring system is additionally optionally substituted with up to three substituents independently selected from WR ^W. 64. The compound of claim 63, wherein G ₄ is a hydrogen atom, G ₅ is a (C ₁ -C ₆ ) aliphatic group, wherein the above aliphatic group is optionally substituted with (C ₁ -C ₆ ) alkyl, a halogen atom, a cyano group, or CF ₃ and where up to two methylene groups of the above (C ₁ -C ₆ ) aliphatic group or (C ₁ -C ₆ ) alkyl are optionally replaced by -CO-, -CONR'-, -CO ₂ -, -OCO-, -NR 'CO ₂ -, -O-, -NR'CONR', -OCONR'-, -NR'CO-, -S-, -NR'-, -SO ₂ NR'-, -NR'SO ₂ - or - NR'SO ₂ NR'-. 65. The compound of claim 63, wherein G ₄ is a hydrogen atom and G ₅ is a cyano group, methyl, ethyl, propyl, isopropyl, butyl, sec-butyl, tert-butyl, cyanomethyl, methoxyethyl, CH ₂ C (O) Me , (CH ₂ ) -NHC (O) O-tert-butyl or cyclopentyl. 66. The compound of claim 63, wherein G ₅ is a hydrogen atom and G ₄ is a halogen atom, a (C ₁ -C ₆ ) aliphatic group or phenyl, wherein the above aliphatic group or phenyl is optionally substituted (substituted) (C ₁ -C ₆ ) alkyl, a halogen atom, a cyano group or CF ₃ , and where up to two methylene groups of the above (C ₁ -C ₆ ) aliphatic group or (C ₁ -C ₆ ) alkyl are optionally replaced by -CO-, -CONR'-, -CO ₂ -, -OCO-, -NR'CO ₂ -, -O-, -NR'CONR ', -OCONR'-, -NR'CO-, -S-, -NR'-, -SO ₂ NR '-, -NR'SO ₂ - or -NR'SO ₂ NR'-. 67. The compound of claim 63, wherein G ₅ is a hydrogen atom, G ₄ is a halogen atom, CF ₃ , ethoxycarbonyl, tert-butyl, 2-methoxyphenyl, 2-ethoxyphenyl, 4-C (O) NH (CH ₂ ) ₂ -Ne ₂ , 2-methoxy-4-chlorophenyl, pyridin-3-yl, 4-isopropylphenyl, 2,6-dimethoxyphenyl, sec-butylaminocarbonyl, ethyl, tert-butyl or piperidin-1-ylcarbonyl. 68. A pharmaceutical composition comprising a compound of formula (I) according to claim 1 and a pharmaceutically acceptable carrier or adjuvant. 69. The composition according to p, where the above composition contains an additional agent selected from a mucolytic agent, bronchodilator agent, antibiotic, anti-infective agent, anti-inflammatory agent, CFTR modulator, or a nutrition-related agent. 70. A method of modulating the activity of the ABC conveyor, comprising the step of contacting the aforementioned ABC conveyor with a compound of formula ( I )

or its pharmaceutically acceptable salt, where Ar ¹ means a 5-6 membered aromatic monocyclic ring having 0-4 heteroatoms independently selected from nitrogen, oxygen or sulfur atoms, where the above ring is optionally fused to a 5-12 membered monocyclic or bicyclic, aromatic, partially unsaturated or saturated ring where each ring contains 0-4 heteroatoms independently selected from nitrogen, oxygen or sulfur atoms, where Ar ¹ has m substituents, each independently selected from —WR ^W ; W means a bond or an optionally substituted (C ₁ -C ₆ ) alkylidene chain, where up to two methylene groups of the W residue are optionally and independently replaced by -CO-, -CS-, -COCO-, -CONR'-, -CONR'NR ', -CO ₂ -, -OCO-, -NR'CO ₂ -, -O-, -NR'CONR'-, -OCONR'-, -NR'NR'-, -NR'NR'CO-, - NR'CO-, -S-, -SO-, -SO ₂ -, -NR'-, -SO ₂ NR'-, -NR'SO ₂ -, -NR'SO ₂ NR'-; R ^W independently means R ', a halogen atom, NO ₂ , CN, CF ₃ or OCF ₃ ; m is 0-5; each of R ¹ , R ² , R ³ , R ⁴ and R ⁵ independently means —X — R ^X ; X is a bond or an optionally substituted (C ₁ -C ₆ ) alkylidene chain, where up to two methylene groups of the X residue are optionally and independently replaced by -CO-, -CS-, -COCO-, -CONR'-, -CONR'NR ', -CO ₂ -, -OCO-, -NR'CO ₂ -, -O-, -NR'CONR'-, -OCONR'-, -NR'NR'-, -NR'NR'CO-, - NR'CO-, -S-, -SO-, -SO ₂ -, -NR'-, -SO ₂ NR'-, -NR'SO ₂ - or -NR'SO ₂ NR'-; R ^X independently means R ', a halogen atom, NO ₂ , CN, CF ₃ or OCF ₃ ; R ⁶ means a hydrogen atom, CF ₃ , -OR ', -SR' or an optionally substituted (C ₁ -C ₈ ) aliphatic group; R ⁷ means a hydrogen atom or a (C ₁ -C ₆ ) aliphatic group optionally substituted with —X — R ^X ; R 'is independently selected from a hydrogen atom or an optionally substituted group selected from a (C ₁ -C ₈ ) aliphatic group, a 3-8 membered saturated, partially unsaturated, or fully unsaturated monocyclic ring having 0-3 heteroatoms independently selected from nitrogen atoms oxygen or sulfur, or an 8-12 membered saturated, partially unsaturated or completely unsaturated bicyclic ring system having 0-5 heteroatoms independently selected from nitrogen, oxygen or sulfur atoms; or two existing R 'radicals taken together with the atom (s) to which they are bonded form an optionally substituted 3-12 membered saturated, partially unsaturated or completely unsaturated monocyclic or bicyclic ring having 0-4 heteroatoms, independently selected from nitrogen, oxygen or sulfur atoms. 71. The method according to item 70, where the above ABC conveyor is CFTR. 72. The method according to item 70, where the modulation of the activity of the ABC transporter is used to treat or reduce the severity of the disease in a patient, where the above disease is selected from cystic fibrosis, hereditary emphysema, hereditary hemochromatosis; coagulation-fibrinolysis deficiencies, such as protein C deficiency, hereditary type 1 angioedema; lipid processing deficits, such as familial hypercholesterolemia, type 1 chylomicronemia, abetalipoproteinemia; lysosomal accumulation diseases such as I-cell inclusions disease / Hurler's pseudosyndrome, mucopolysaccharidoses, Sandhoff / Tey-Sachs disease, type II Kriegler-Nayyar disease, polyendocrinopathy / hyperinsulemia, diabetes mellitus, Laron microsomy, glomerosicanosis, mileopenosis deficiency, meloperosanosis, Type 1 CDG, congenital hyperthyroidism, osteopsatyrosis, hereditary hypofibrogenemia, adrenocorticotropic hormone deficiency, diabetes insipidus (DI), neurophysical DI, non-progenic DI, Charcot-Marie-Tooth syndrome, Per disease itseusa-Merzbacher; neurodegenerative diseases such as Alzheimer's disease, Parkinson's disease, amphiotrophic lateral sclerosis, progressive supranuclearplasia, Peak's disease; severe polyglutamine neurological disorders, such as Huntington’s disease, type I spinocerebral ataxia, spinal and bulbar muscular atrophy, dentatorubal pallidoluysian and myotonic dystrophy, as well as spongiform encephalopathies, such as hereditary Kreutzfeldt-Jacob disease, Straussler-Scheinker syndrome, COPD, dry eye disease, or Sjogren's syndrome; comprising the step of administering to the above patient an effective amount of a compound of formula ( I ) according to claim 70. 73. A kit for use in measuring the activity of an ABC transporter or fragment thereof in an in vitro or in vivo biological sample, including (i) a composition comprising a compound of formula ( I ) according to claim 70; (ii) instructions for: a) contacting the composition with a biological sample; b) measuring the activity of the aforementioned ABC transporter or fragment thereof. 74. The kit of claim 73, further comprising instructions for: a) contacting the additional composition with a biological sample; b) measuring the activity of the aforementioned ABC transporter or fragment thereof in the presence of an additional compound; and c) comparing the activity of the ABC transporter in the presence of said additional compound with the density of the ABC transporter in the presence of a compound of formula ( I ). 75. The kit of claim 74, wherein the kit is used in measuring CFTR density.