RU2009141996A - ATF-BINDING CASSETTE MODULATORS - Google Patents

Abstract

 1. The compound of formula II! ! II! or its pharmaceutically acceptable salt, where:! each of WRW2 and WRW4 is independently selected from —CN, —CF3, a halogen atom, a linear or branched (C2-C6) alkyl, (C3-C12) membered cycloaliphatic group, phenyl, a 5-10 membered heteroaryl, or 3-7- a heterocyclic group, wherein said heteroaryl or said heterocyclic group has up to 3 heteroatoms selected from O, S or N, where said WRW2 and WRW4 are independently and optionally substituted with up to three substituents selected from -OR ', -CF3, -OCF3 , SR ', S (O) R', SO2R ', -SCF3, halogen atom, CN, -COOR', -COR ', -O (CH2) 2N (R') 2, -O (CH2) N (R ') 2, -CON (R') 2, - (CH2) 2OR ', - (CH2) OR', -CH2CN, optionally substituted phenyl or phenoxy groups, -N (R ') 2, -NR'C (O) OR', -NR'C (O) R ', - (CH2) 2N (R') 2 or - (CH2) N ( R ') 2; ! WRW5 is selected from hydrogen, —OCF3, —CF3, —OH, —OCH3, —NH2, —CN, —CHF2, —NHR ′, —N (R ′) 2, —NHC (O) R ′, —NHC (O ) OR ', -NHSO2R', -CH2OH, -CH2N (R ') 2, -C (O) OR', -SO2NHR ', -SO2N (R') 2 or -CH2NHC (O) OR '; and! each R ′ is independently selected from an optionally substituted group selected from a (C1-C8) aliphatic group, a 3-8 membered saturated, partially unsaturated, or fully unsaturated monocyclic ring having 0-3 heteroatoms independently selected from nitrogen, oxygen, or sulfur atoms 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 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; ! under the condition

Claims (46)

1. The compound of formula II

II or its pharmaceutically acceptable salt, where: each of WR ^W2 and WR ^{W4 is} independently selected from —CN, —CF ₃ , a halogen atom, a linear or branched (C ₂ -C ₆ ) alkyl, (C ₃ -C ₁₂ )-membered cycloaliphatic group, phenyl, 5-10- heteroaryl or a 3-7 membered heterocyclic group, wherein said heteroaryl or said heterocyclic group has up to 3 heteroatoms selected from O, S or N, where said 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 ') ₂ , -O (CH ₂ ) N (R') ₂ , -CON (R ') ₂ , - (CH ₂ ) ₂ OR', - (CH ₂ ) OR ', -CH ₂ CN, optionally substituted phenyl or phenoxy groups, -N (R') ₂ , -NR'C (O) OR ', -NR'C (O) R', - (CH ₂ ) ₂ N (R ') ₂ or - (CH ₂ ) N (R') ₂ ; WR ^{W5 is} selected from hydrogen, —OCF ₃ , —CF ₃ , —OH, —OCH ₃ , —NH ₂ , —CN, —CHF ₂ , —NHR ′, —N (R ′) ₂ , —NHC (O) R ', -NHC (O) OR', -NHSO ₂ R ', -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'; and each R ′ is independently selected from 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, 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; with the proviso that the compound of formula II is not N- (5-hydroxy-2,4-di-tert-butylphenyl) -4-oxo-1H-quinoline-3-carboxamide or 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 each of WR ^W2 and WR ^{W4 is} independently selected from CN, CF ₃ , a halogen atom, a linear or branched (C ₂ -C ₆ ) alkyl, (C ₃ -C ₁₂ ) -membered cycloaliphatic group or phenyl, wherein said WR ^W2 and WR ^{W4 are} 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 ') ₂ , -O (CH ₂ ) N (R') ₂ , -CON (R ') ₂ , - (CH ₂ ) ₂ OR', - (CH ₂ ) OR ', optionally substituted phenyl, —N (R ′) ₂ , —NC (O) OR ′, —NC (O) R ′, - (CH ₂ ) ₂ N (R ′) _2, or - (CH ₂ ) N (R ′ ) ₂ ; and WR ^{W5 is} selected from hydrogen, —OCF ₃ , —CF ₃ , —OH, —OCH ₃ , —NH ₂ , —CN, —NHR ′, —N (R ′) ₂ , —NHC (O) R ′, —NHC (O) OR ', -NHSO ₂ R', -CH ₂ OH, -C (O) OR ', -SO ₂ NHR' or -CH ₂ NHC (O) O- (R '). 3. The compound according to claim 2, where each of WR ^W2 and WR ^{W4 is} independently selected from CN, CF ₃ , linear or branched (C ₂ -C ₆ ) alkyl, (C ₃ -C ₁₂ ) -membered cycloaliphatic group or phenyl, where each of said WR ^W2 and WR ^{W4 is} 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 ') ₂ , -O (CH ₂ ) N (R') ₂ , -CON (R ') ₂ , - (CH ₂ ) ₂ OR', - (CH ₂ ) OR ', optional substituted phenyl, -N (R ') ₂ , -NC (O) OR', -NC (O) R ', - (CH ₂ ) ₂ N (R') ₂ or - (CH ₂ ) N (R ') ₂ ; and WR ^{W5 is} selected from —OH, —CN, —NHR ′, —N (R ′) ₂ , —NHC (O) R ′, —NHC (O) OR ′, —NHSO ₂ R ′, —CH ₂ OH, - C (O) OR ', -SO ₂ NHR' or -CH ₂ NHC (O) O- (R '). 4. The compound of claim 3, wherein WR ^W2 is 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') ₂ , -O (CH ₂ ) N (R ') ₂ , - CON (R ') ₂ , - (CH ₂ ) ₂ OR', - (CH ₂ ) OR ', -CH ₂ CN, optionally substituted phenyl or phenoxy, -N (R') ₂ , -NR'C (O) OR ', -NR'C (O) R', - (CH ₂ ) ₂ N (R ') ₂ or - (CH ₂ ) N (R') ₂ ; WR ^W4 is linear or branched (C ₂ -C ₆ ) alkyl; and WR ^W5 means OH. 5. The compound according to claim 3, where each of WR ^W2 and WR ^{W4 are} independently selected from CF ₃ , CN, or linear or branched (C ₂ -C ₆ ) alkyl. 6. The compound according to claim 5, where each of WR ^W2 and WR ^W4 means a linear or branched (C ₂ -C ₆ ) alkyl, optionally substituted with up to three substituents independently 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') ₂ , —O (CH ₂ ) N (R ′) ₂ , —CON (R ′) ₂ , - (CH ₂ ) ₂ OR ′, - (CH ₂ ) OR ′, —CH ₂ CN, optionally substituted phenyl or phenoxy groups, -N (R ') ₂ , -NR'C (O) OR', -NR'C (O) R ', - (CH ₂ ) ₂ N (R') ₂ or - (CH ₂ ) N (R ' ) ₂ . 7. The compound according to claim 6, where 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. 8. The compound according to claim 3, where WR ^{W5 is} selected from —CN, —NHR ′, —N (R ′) ₂ , —CH ₂ N (R ′) ₂ , —NHC (O) R ′, —NHC (O ) OR ', -OH, C (O) OR' or -SO ₂ NHR '. 9. The compound of claim 8, where WR ^{W5 is} selected from —CN, —NH (C ₁ -C ₆ ) alkyl, —N ((C ₁ -C ₆ ) alkyl) ₂ , —NHC (O) (C ₁ - C ₆ ) alkyl, -CH ₂ NHC (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 . 10. The compound according to claim 9, where WR ^{W5 is} selected from —OH, —CH ₂ OH, —NHC (O) OMe, —NHC (O) OEt, —CN, —CH ₂ NHC (O) O (tert-butyl ), -C (O) OMe or -SO ₂ NH ₂ . 11. The compound according to claim 1, where a. WR ^W2 is straight or branched (C ₂ -C ₆ ) alkyl; b. WR ^W4 means a linear or branched (C ₂ -C ₆ ) alkyl or a monocyclic or bicyclic aliphatic group; and c. WR ^{W5 is} selected from —CN, —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 . 12. The compound according to claim 1, where a. WR ^W2 means (C ₂ -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 an atom halogen; b. WR ^W4 is CF ₃ , (C ₂ -C ₆ ) alkyl or a (C ₆ -C ₁₀ ) cycloaliphatic group; and c. WR ^W5 is —OH, —NH (C ₁ –C ₆ ) alkyl, or —N ((C ₁ –C ₆ ) alkyl) ₂ . 13. The compound according to claim 1, where WR ^W2 means tert-butyl. 14. The compound of claim 13, wherein WR ^W4 is tert-butyl. 15. The compound of claim 14, wherein WR ^W5 is —OH. 16. The compound of formula III

b III or its pharmaceutically acceptable salt, where: X is a bond or an optionally substituted linear or branched (C ₁ -C ₆ ) alkylidene chain, where up to two methylene groups of the residue X are optionally and independently replaced by -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'-; each R ^X independently means R ', a halogen atom, —NO ₂ , —CN, —CF ₃ or —OCF ₃ ; each R ′ is independently selected from hydrogen, a halogen 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, oxygen or sulfur atoms, 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 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; m is an integer from 0 to 6; WR ^W2 is a linear or branched (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; WR ^W4 is a linear or branched (C ₁ -C ₆ ) alkyl, monocyclic or bicyclic aliphatic group or —CF ₃ ; and WR ^{W5 is} selected from —CN, —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 , with the proviso that the compound of formula III is not N- (5-hydroxy-2,4-di-tert-butylphenyl) -4-oxo-1H-quinoline-3-carboxamide or a pharmaceutically acceptable salt of N- (5-hydroxy-2 , 4-di-tert-butylphenyl) -4-oxo-1H-quinoline-3-carboxamide. 17. The compound according to clause 16, where m is from 1 to 6, X is a bond, R ^X is hydrogen, a halogen atom, —NO ₂ , —CN, —CF ₃ , —OCF ₃ , —OH, or optionally substituted (C ₁ -C ₆ ) an aliphatic group. 18. The compound according to 17, where m means from 1 to 6, X is independently selected from a bond or an optionally substituted linear (C ₁ -C ₃ ) alkylidene chain, R ^{X is} independently selected from an optionally substituted (C ₃ -C ₈ ) monocyclic or a bicyclic bicyclic cycloaliphatic group. 19. The compound of claim 16, wherein m is from 2 to 6, X is a bond, R ^{X is} independently selected from hydrogen, a halogen atom, —NO ₂ , —CN, —CF ₃ , —OCF ₃ , —OH, or optionally substituted (C ₁ -C ₆ ) aliphatic group. 20. The compound according to claim 19, where m is from 3 to 6, X is a bond, R ^{X is} independently selected from hydrogen, a halogen atom, —NO ₂ , —CN, —CF ₃ , —OCF ₃ , —OH, or optionally substituted (C ₁ -C ₆ ) aliphatic group. 21. The compound according to claim 20, where m is from 4 to 6, X is a bond, R ^{X is} independently selected from hydrogen, a halogen atom, —NO ₂ , —CN, —CF ₃ , —OCF ₃ , —OH, or optionally substituted (C ₁ -C ₆ ) aliphatic group. 22. The compound according to item 21, where m is 5, X is a bond, R ^{X is} independently selected from hydrogen, a halogen atom, —NO ₂ , —CN, —CF ₃ , —OCF ₃ , —OH, or optionally substituted (C ₁ -C ₆ ) an aliphatic group. 23. The compound of claim 22, where m is 6, X is a bond, R ^{X is} independently selected from hydrogen, a halogen atom, —NO ₂ , —CN, —CF ₃ , —OCF ₃ , —OH, or optionally substituted (C ₁ -C ₆ ) an aliphatic group. 24. The compound of claim 16, wherein at least one of XR ^X is hydrogen. 25. The compound of claim 24, wherein at least two of XR ^{X are} hydrogen. 26. The compound of claim 25, wherein at least three of XR ^{X are} hydrogen. 27. The compound of claim 26, wherein at least four of XR ^{X are} hydrogen. 28. The compound of claim 27, wherein at least five of XR ^{X are} hydrogen. 29. The compound of claim 28, wherein all six of XR ^{X are} hydrogen. 30. The compound according to any one of paragraphs.16-29, where a. WR ^W2 is linear or branched (C ₁ -C ₆ ) alkyl; b. WR ^W4 means a linear or branched (C ₁ -C ₆ ) alkyl or a monocyclic or bicyclic aliphatic group; and c. WR ^{W5 is} selected from —CN, —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 any one of paragraphs.16-29, where a. WR ^W2 means (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 , (C ₁ -C ₆ ) alkyl or a (C ₆ -C ₁₀ ) cycloaliphatic group; and c. WR ^W5 is OH, —NH (C ₁ -C ₆ ) alkyl, or —N ((C ₁ -C ₆ ) alkyl) ₂ . 32. The compound according to any one of paragraphs.16-29, where WR ^W2 means tert-butyl. 33. The compound according to any one of paragraphs.16-29, where WR ^W4 means tert-butyl. 34. The compound according to any one of claims 16 to 29, wherein WR ^W5 is —OH. 35. A pharmaceutical composition comprising a compound of formula II according to claim 1 and a pharmaceutically acceptable carrier or adjuvant. 36. The composition of claim 35, wherein the above composition comprises an additional agent selected from a mucolytic agent, a bronchodilator, an antibiotic, an anti-infective agent, an anti-inflammatory agent, a CFTR modulator, or a nutrition-related agent. 37. A pharmaceutical composition comprising a compound of formula III according to claim 16 and a pharmaceutically acceptable carrier or adjuvant. 38. The composition according to clause 37, where the above composition contains an additional agent selected from a mucolytic agent, bronchodilator agent, antibiotic, anti-infectious agent, anti-inflammatory agent, CFTR modulator, or a food-related agent. 39. A method of modulating the activity of an ABC transporter comprising the step of contacting the aforementioned ABC transporter with a compound of formula II

II or its pharmaceutically acceptable salt, where: each of WR ^W2 and WR ^{W4 is} independently selected from —CN, —CF ₃ , a halogen atom, a linear or branched (C ₁ -C ₆ ) alkyl, (C ₃ -C ₁₂ )-membered cycloaliphatic group, phenyl, 5-10- heteroaryl or a 3-7 membered heterocyclic group, wherein said heteroaryl or said heterocyclic group has up to 3 heteroatoms selected from O, S or N, where said 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 ') ₂ , -O (CH ₂ ) N (R') ₂ , -CON (R ') ₂ , - (CH ₂ ) ₂ OR', - (CH ₂ ) OR ', -CH ₂ CN, optionally substituted phenyl or phenoxy groups, -N (R') ₂ , -NR'C (O) OR ', -NR'C (O) R', - (CH ₂ ) ₂ N (R ') ₂ or - (CH ₂ ) N (R') ₂ ; WR ^{W5 is} selected from hydrogen, —OCF ₃ , —CF ₃ , —OH, —OCH ₃ , —NH ₂ , —CN, —CHF ₂ , —NHR ′, —N (R ′) ₂ , —NHC (O) R ', -NHC (O) OR', -NHSO ₂ R ', -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'; and each R ′ is independently selected from 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, 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. 40. The method according to § 39, where the aforementioned ABC-conveyor is CFTR. 41. A method for modulating the activity of an ABC transporter comprising the step of contacting the aforementioned ABC transporter with a compound of formula III

III or its pharmaceutically acceptable salt, where: X is a bond or an optionally substituted linear or branched (C ₁ -C ₆ ) alkylidene chain, where up to two methylene groups of the residue X are optionally and independently replaced by -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'-; each R ^X independently means R ', a halogen atom, —NO ₂ , —CN, —CF ₃ or —OCF ₃ ; each R ′ is independently selected from hydrogen, a halogen 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, oxygen or sulfur atoms, 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 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; m is an integer from 0 to 6; WR ^W2 is a linear or branched (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; WR ^W4 is a linear or branched (C ₁ -C ₆ ) alkyl, monocyclic or bicyclic aliphatic group or —CF ₃ ; and WR ^{W5 is} selected from —CN, —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 . 42. The method according to paragraph 41, where the aforementioned ABC-conveyor is CFTR. 43. The method according to § 39 or 41, 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, osteopsatirosis, 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 II according to claim 1 or a compound of formula III according to clause 16. 44. 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 (II) according to claim 1; or (ii) a composition comprising a compound of formula (III) according to claim 16; and (iii) instructions for: a) contacting the composition with a biological sample; b) measuring the activity of the aforementioned ABC transporter or fragment thereof. 45. The kit according to item 44, 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 said additional compound; and c) comparing the activity of the ABC transporter in the presence of an additional compound with the density of the ABC transporter in the presence of a composition of formula (II) or a composition of formula (III). 46. The kit according to item 45, where the kit is used in the measurement of CFTR density.