Direct interaction of a cftr potentiator and a cftr corrector with phospholipid bilayers

European Biophysics Journal

Volumn 43, Issue 6-7, 2014, Pages 341-346

  Baroni, Debora ^a   Zegarra Moran, Olga ^b   Svensson, Agneta ^c   Moran, Oscar ^a  

^a CNR   (Italy)

^b G GASLINI INSTITUTE   (Italy)

^c ALBA SYNCHROTRON LIGHT SOURCE   (Spain)

Author keywords

Bilayer; Corrector; Cystic fibrosis transmembrane conductance regulator (CFTR); Potentiator; Small angle X ray scattering

Indexed keywords

CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; IVACAFTOR; LUMACAFTOR; 1,3 BENZODIOXOLE DERIVATIVE; AMINOPHENOL DERIVATIVE; AMINOPYRIDINE DERIVATIVE; LIPID BILAYER; LIPOSOME; N-(2,4-DI-TERT-BUTYL-5-HYDROXYPHENYL)-4-OXO-1,4-DIHYDROQUINOLINE-3-CARBOXAMIDE; PHOSPHOLIPID; PROTEIN BINDING; QUINOLONE DERIVATIVE;

ARTICLE; CONTROLLED STUDY; DRUG PENETRATION; MEMBRANE PERMEABILITY; PHOSPHOLIPID BILAYER; PRIORITY JOURNAL; SYNCHROTRON RADIATION; THERMOSTABILITY; X RAY CRYSTALLOGRAPHY; CHEMISTRY; LIPID BILAYER; METABOLISM; TEMPERATURE;

AMINOPHENOLS; AMINOPYRIDINES; BENZODIOXOLES; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; LIPID BILAYERS; PHOSPHOLIPIDS; PROTEIN BINDING; QUINOLONES; TEMPERATURE; UNILAMELLAR LIPOSOMES;

EID: 84905719391     PISSN: 01757571     EISSN: 14321017     Source Type: Journal    
DOI: 10.1007/s00249-014-0956-y     Document Type: Article

References (18)

1. Bobadilla JL, Macek MJ, Fine JP, Farrell PM (2002) Cystic fibrosis: a worldwide analysis of CFTR mutations-correlation with incidence data and application to screening. Hum Mutat 19:575-606
2. Brzustowicz MR, Brunger AT (2005) X-ray scattering from unilamellar lipid vesicles. J Appl Cryst 38:126-131
3. Dalemans W, Barbry P, Champigny G, Jallat S, Dott K, Dreyer D, Crystal RG, Pavirani A, Lecocq JP, Lazdunski M (1991) Altered chloride ion channel kinetics associated with the delta F508 cystic fibrosis mutation. Nature 354:526-528
4. Gianotti A, Melani R, Caci E, Sondo E, Ravazzolo R, Galietta LJV, Zegarra-Moran O (2013) Epithelial sodium channel silencing as a strategy to correct the airway surface fluid deficit in cystic fibrosis. Am J Respir Cell Mol Biol 49:445-452
5. Hammersley AP, Svensson SO, Hanfland M, Fitch AN, Hausermann D (1996) Two-dimensional detector software: from real detector to idealised image or two-theta scan. High Press Res 14:248-325
6. Haydon DA, Hendry BM, Levinson SR, Requena J (1977) The molecular mechanisms of anaesthesia. Nature 268:356-358
7. Hirai M, Iwase H, Hayakawa T, Koizumi M, Takahashi H (2003) Determination of asymmetric structure of ganglioside-DPPC mixed vesicle using SANS, SAXS, and DLS. Biophys J 85:1600-1610
8. Kucerka N, Pencer J, Sachs JN, Nagle JF, Katsaras J (2007) Curvature effect on the structure of phospholipid bilayers. Langmuir 23:1292-1299
9. Lewis BA, Engelman DM (1983) Lipid bilayer thickness varies linearly with acyl chain length in fluid phosphatidylcholine vesicles. J Mol Biol 166:211-217
10. Lopes LB, Scarpa MV, Silva GVJ, Rodrigues DC, Santilli CV, Oliveira AG (2004) Studies on the encapsulation of diclofenac in small unilamellar liposomes of soya phosphatidylcholine. Colloids Surf B Biointerfaces 39:151-158
11. Lukacs GL, Mohamed A, Kartner N, Chang XB, Riordan JR, Grinstein S (1994) Conformational maturation of CFTR but not its mutant counterpart (delta F508) occurs in the endoplasmic reticulum and requires ATP. EMBO J 13:6076-6086
12. MacDonald RC, MacDonald RI, Menco BP, Takeshita K, Subbarao NK, Hu LR (1991) Small-volume extrusion apparatus for preparation of large, unilamellar vesicles. Biochim Biophys Acta 1061:297-303
13. Mateu L, Moran O (1986) Reversible changes in myelin structure and electrical activity during anesthesia in vivo. Biochim Biophys Acta 862:17-26
14. Moran O, Galietta LJV, Zegarra-Moran O (2005) Binding site of activators of the cystic fibrosis transmembrane conductance regulator in the nucleotide binding domains. Cell Mol Life Sci 62:446-460
15. Pabst G, Rappolt M, Amenitsch H, Laggner P (2000) Structural information from multilamellar liposomes at full hydration: full q-range fitting with high-quality X-ray data. Phys Rev E62:4000-4009
16. Ramsey BW, Davies J, McElvaney NG, Tullis E, Bell SC, Drevinek P, Griese M, McKone EF, Wainwright CE, Konstan MW, Moss R, Ratjen F, Sermet-Gaudelus I, Rowe SM, Dong Q, Rodriguez S, Yen K, Ordonez C, Elborn JS (2011) A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N Engl J Med 365:1663-1672
17. Su C, Wu S, Jeng U, Lee M, Su A, Liao K, Lin W, Huang Y, Chen C (2013) Peptide-induced bilayer thinning structure of unilamellar vesicles and the related binding behavior as revealed by X-ray scattering. Biochim Biophys Acta 1828:528-534
18. Van Goor F, Hadida S, Grootenhuis PDJ, Burton B, Stack JH, Straley KS, Decker CJ, Miller M, McCartney J, Olson ER, Wine JJ, Frizzell RA, Ashlock M, Negulescu PA (2011) Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809. Proc Natl Acad Sci USA 108:18843-18848