Pharmacotherapy of the ion transport defect in cystic fibrosis

Clinical and Experimental Pharmacology and Physiology

Volumn 28, Issue 11, 2001, Pages 857-867

  Kunzelmann, Karl ^a   Mall, Marcus ^b  

^a UNIVERSITY OF QUEENSLAND   (Australia)

^b UNIVERSITY OF FREIBURG   (Germany)

Author keywords

8 cyclopentyl 1,3 dipropylxanthine; Amiloride; Benzimidazol; Chaperone; Cystic fibrosis; Cystic fibrosis transmembrane conductance regulator; Deltametrin; Epithelial Na+ channel; Genistein; Phenylbutyrate; Psoralen

Indexed keywords

1 (5 CHLORO 2 HYDROXYPHENYL) 5 TRIFLUOROMETHYL 2 BENZIMIDAZOLONE; 1 ETHYL 2 BENZIMIDAZOLINONE; 4 PHENYLBUTYRIC ACID; 8 CYCLOPENTYL 1,3 DIPROPYLXANTHINE; AMILORIDE; AMINOGLYCOSIDE; AMRINONE; BENZAMIL; BENZIMIDAZOLE DERIVATIVE; BUTYRIC ACID; CALCIUM; CHLORIDE; CHLORIDE CHANNEL; CHLORZOXAZONE; DIQUAFOSOL; DURAMYCIN; GENISTEIN; GENTAMICIN; GLYCEROL; ISOBUTYLMETHYLXANTHINE; LOPERAMIDE; MILRINONE; PHENAMIL; POTASSIUM; POTASSIUM CHANNEL; PURINE P2Y RECEPTOR; PURINERGIC RECEPTOR STIMULATING AGENT; SODIUM; SODIUM CHANNEL; SODIUM CHANNEL BLOCKING AGENT; SODIUM CHLORIDE; TRANSMEMBRANE CONDUCTANCE REGULATOR; TRIMETHYLAMINE OXIDE; UNCLASSIFIED DRUG; UNINDEXED DRUG;

CHLORIDE TRANSPORT; CLINICAL TRIAL; CYSTIC FIBROSIS; DISEASE COURSE; DRUG MECHANISM; GENE ACTIVATION; GENE MUTATION; HUMAN; IN VIVO STUDY; ION CONDUCTANCE; ION TRANSPORT; LUNG DISEASE; NONHUMAN; PROTEIN EXPRESSION; PROTEIN PROCESSING; PROTEIN SYNTHESIS; REGULATORY MECHANISM; REVIEW; SODIUM ABSORPTION; TRANSCRIPTION REGULATION;

EID: 0035163461     PISSN: 03051870     EISSN: None     Source Type: Journal    
DOI: 10.1046/j.1440-1681.2001.03541.x     Document Type: Review

References (70)

1. Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA
2. CFTR is a conductance regulator as well as a chloride channel
3. The cystic fibrosis transmembrane conductance regulator and its function in epithelial transport
4. New paradigms of CFTR chloride channel regulation
5. Type I, II, III, IV, and V cystic fibrosis transmembrane conductance regulator defects and opportunities for therapy
6. Pharmacological treatment of the ion transport defect in cystic fibrosis
7. Future pharmacological treatment of cystic fibrosis
8. CFTR, a regulator of channels
9. Genotype and phenotype in cystic fibrosis
10. Epithelial sodium channels: Function, structure and regulation
11. Pharmacologic treatment of abnormal ion transport in the airway epithelium in cystic fibrosis
12. A pilot study of aerosolized amiloride for the treatment of lung disease in cystic fibrosis
13. No added benefit from nebulized amiloride in patients with cystic fibrosis
14. Effects of topically delivered benzamil and amiloride on nasal potential difference in cystic fibrosis
15. + absorption by extra-cellular nucleotides in human normal and CF airways
16. + absorption in wild-type and DeltaF508 CFTR-expressing human bronchial epithelia
17. 2-receptor agonists enhances mucociliary clearance in sheep
18. + channels in respiratory epithelia
19. Chloride secretory response to extracellular ATP in human normal and cystic fibrosis nasal epithelia
20. Activation by extracellular nucleotides of chloride secretion in the airway epithelia of patients with cystic fibrosis
21. Effect of extracellular ATP and UTP on fluid transport by human nasal epithelial cells in culture
22. Duramycin enhances chloride secretion in airway epithelium
23. Stimulation of sodium transport by duramycin in cultured human colonic epithelia
24. - secretion in human airways
25. - secretion by chlorzoxazone
26. + channel
27. + secretion in rectal biopsies from cystic fibrosis patients
28. Degradation of CFTR by the ubiquitin-proteasome pathway
29. Multiple proteolytic systems, including the proteasome, contribute to CFTR processing
30. Aminoglycoside antibiotics restore CFTR function by overcoming premature stop mutations
31. A pilot study of the effect of gentamicin on nasal potential difference measurements in cystic fibrosis patients carrying stop mutations
32. Sodium 4-phenylbutyrate downregulates Hsc70: Implications for intracellular trafficking of DeltaF508-CFTR
33. A pilot clinical trial of oral sodium 4-phenylbutyrate (Buphenyl) in deltaF508-homozygous cystic fibrosis patients: Partial restoration of nasal epithelial CFTR function
34. Butyrate increases apical membrane CFTR but reduces chloride secretion in MDCK cells
35. - channel by butyrate and phenylbutyrate
36. Glycerol reverses the misfolding phenotype of the most common cystic fibrosis mutation
37. Defects in processing and trafficking of CFTR
38. BAP31 dependent expression of CFTR
39. Partial restoration of cAMP-stimulated CFTR chloride channel activity in DeltaF508 cells by deoxyspergualin
40. Post-translational disruption of the delta F508 CFTR-molecular chaperone complex with geldanamycin stabilises delta F508 CFTR in the rabbit reticulocyte lysate
41. Biosynthesis and degradation of CFTR
42. CFTR-mediated chloride permeability is regulated by type III phosphodiesterases in airway epithelial cells
43. The in vivo effects of milrinone on the airways of cystic fibrosis mice and human subjects
44. In vivo activation of the cystic fibrosis transmembrane conductance regulator mutant deltaF508 in murine nasal epithelium
45. Pharmacology of CFTR chloride channel activity
46. Phosphatase inhibitors activate normal and defective CFTR chloride channels
47. 2+-independent activation of cystic fibrosis transmembrane conductance regulator channels by phenylimidazothiazole drugs
48. Role of protein phosphatases in the activation of CFTR (ABCC7) by genistein and bromotetramisole
49. + channel blockade
50. Activation of deltaF508 CFTR in a cystic fibrosis respiratory epithelial cell line by 4-phenylbutyrate, genistein and CPX
51. Stimulation by alkylxanthines of chloride efflux in CFPAC-1 cells does not involve A1 adenosine receptors
52. 8-cyclopentyl-1,3-dipropylxanthine and other xanthines differentially bind to the wild-type and deltaF508 mutant first nucleotide binding fold (NBF-1) domains of the cystic fibrosis transmembrane conductance regulator
53. Direct activation of cystic fibrosis transmembrane conductance regulator channels by 8-cyclopentyl-1,3-dipropylxanthine (CPX) and 1,3-diallyl-8-cyclohexylxanthine (DAX)
54. No evidence for direct activation of CFTR by 8-cyclopentyl-1,3-dipropylxanthine (CPX)
55. Potent stimulation and inhibition of the CFTR Cl (-) current by phloxine B
56. - channels by the tyrosine kinase inhibitor genistein
57. Genistein potentiates wild-type and delta F508-CFTR channel activity
58. Effect of genistein on native epithelial tissues from normal individuals and CF patients and on CFTR expressed in Xenopus oocytes
59. The substituted benzimidazolone NS004 is an opener of the cystic fibrosis chloride channel
60. Pharmacological modulation of ion transport across wild-type and DeltaF508 CFTR-expressing human bronchial epithelia
61. - secretion
62. - conductances in epithelial cells
63. s-Carbocysteinelysine salt monohydrate and cAMP cause non-additive activation of the cystic fibrosis transmembrane regulator channel in human respiratory epithelium
64. Development of substituted benzo[c]quinolizinium compounds as novel activators of the cystic fibrosis chloride channel
65. Novel CFTR chloride channel activators identified by screening of combinatorial libraries based on flavone and benzoquinolizinium lead compounds
66. Effect of uridine 5′-triphosphate plus amiloride on mucociliary clearance in adult cystic fibrosis
67. Acute safety and effects on mucociliary clearance of aerosolized uridine 5′-triphosphate +/--amiloride in normal human adults
68. Sodium channel blockers and uridine triphosphate: Effects on nasal potential difference in cystic fibrosis mice
69. Effects of methacholine and uridine 5′-triphosphate on tracheal mucus rheology in mice
70. - conductance of human airway epithelium in vitro and in vivo