-
1
-
-
0034635458
-
Cystic fibrosis transmembrane conductance regulator. Structure and function of an epithelial chloride channel
-
Akabas M.H. Cystic fibrosis transmembrane conductance regulator. Structure and function of an epithelial chloride channel. J. Biol. Chem. 2000, 275:3729-3732.
-
(2000)
J. Biol. Chem.
, vol.275
, pp. 3729-3732
-
-
Akabas, M.H.1
-
2
-
-
84926365000
-
The fast azimuthal integration Python library: pyFAI
-
Ashiotis G., Deschildre A., Nawaz Z., Wright J.P., Karkoulis D., Picca F.E., Kieffer J. The fast azimuthal integration Python library: pyFAI. J. Appl. Crystallogr. 2015, 48:510-519.
-
(2015)
J. Appl. Crystallogr.
, vol.48
, pp. 510-519
-
-
Ashiotis, G.1
Deschildre, A.2
Nawaz, Z.3
Wright, J.P.4
Karkoulis, D.5
Picca, F.E.6
Kieffer, J.7
-
3
-
-
77951118320
-
Structures of a minimal human CFTR first nucleotide-binding domain as a monomer, head-to-tail homodimer, and pathogenic mutant
-
Atwell S., Brouillette C.G., Conners K., Emtage S., Gheyi T., Guggino W.B., Hendle J., Hunt J.F., Lewis H.A., Lu F., et al. Structures of a minimal human CFTR first nucleotide-binding domain as a monomer, head-to-tail homodimer, and pathogenic mutant. Protein Eng. Des. Sel. 2010, 23:375-384.
-
(2010)
Protein Eng. Des. Sel.
, vol.23
, pp. 375-384
-
-
Atwell, S.1
Brouillette, C.G.2
Conners, K.3
Emtage, S.4
Gheyi, T.5
Guggino, W.B.6
Hendle, J.7
Hunt, J.F.8
Lewis, H.A.9
Lu, F.10
-
4
-
-
27844520938
-
Crystallographic and single-particle analyses of native- and nucleotide-bound forms of the cystic fibrosis transmembrane conductance regulator (CFTR) protein
-
Awayn N.H., Rosenberg M.F., Kamis A.B., Aleksandrov L.A., Riordan J.R., Ford R.C. Crystallographic and single-particle analyses of native- and nucleotide-bound forms of the cystic fibrosis transmembrane conductance regulator (CFTR) protein. Biochem. Soc. Trans. 2005, 33:996-999.
-
(2005)
Biochem. Soc. Trans.
, vol.33
, pp. 996-999
-
-
Awayn, N.H.1
Rosenberg, M.F.2
Kamis, A.B.3
Aleksandrov, L.A.4
Riordan, J.R.5
Ford, R.C.6
-
5
-
-
79958104493
-
Pharmacological therapy for cystic fibrosis: from bench to bedside
-
Becq F., Mall M.A., Sheppard D.N., Conese M., Zegarra-Moran O. Pharmacological therapy for cystic fibrosis: from bench to bedside. J. Cyst. Fibros. 2011, 10(Suppl. 2):S129-S145.
-
(2011)
J. Cyst. Fibros.
, vol.10
, pp. S129-S145
-
-
Becq, F.1
Mall, M.A.2
Sheppard, D.N.3
Conese, M.4
Zegarra-Moran, O.5
-
6
-
-
84922309675
-
On the interactions between nucleotide binding domains and membrane spanning domains in cystic fibrosis transmembrane regulator: a molecular dynamic study
-
Belmonte L., Moran O. On the interactions between nucleotide binding domains and membrane spanning domains in cystic fibrosis transmembrane regulator: a molecular dynamic study. Biochimie 2015, 111:19-29.
-
(2015)
Biochimie
, vol.111
, pp. 19-29
-
-
Belmonte, L.1
Moran, O.2
-
7
-
-
84862524410
-
Modeling detergent organization around aquaporin-using small-angle X-ray scattering
-
Berthaud A., Manzi J., Pérez J., Mangenot S. Modeling detergent organization around aquaporin-using small-angle X-ray scattering. J. Am. Chem. Soc. 2012, 134:10080-10088.
-
(2012)
J. Am. Chem. Soc.
, vol.134
, pp. 10080-10088
-
-
Berthaud, A.1
Manzi, J.2
Pérez, J.3
Mangenot, S.4
-
8
-
-
84904002908
-
A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 randomised controlled trial
-
Boyle M.P., Bell S.C., Konstan M.W., McColley S.A., Rowe S.M., Rietschel E., Huang X., Waltz D., Patel N.R., Rodman D. A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 randomised controlled trial. Lancet Respir. Med. 2014, 2:527-538.
-
(2014)
Lancet Respir. Med.
, vol.2
, pp. 527-538
-
-
Boyle, M.P.1
Bell, S.C.2
Konstan, M.W.3
McColley, S.A.4
Rowe, S.M.5
Rietschel, E.6
Huang, X.7
Waltz, D.8
Patel, N.R.9
Rodman, D.10
-
9
-
-
0032805390
-
A method for determining transmembrane helix association and orientation in detergent micelles using small angle X-ray scattering
-
Bu Z., Engelman D.M. A method for determining transmembrane helix association and orientation in detergent micelles using small angle X-ray scattering. Biophys. J. 1999, 77:1064-1073.
-
(1999)
Biophys. J.
, vol.77
, pp. 1064-1073
-
-
Bu, Z.1
Engelman, D.M.2
-
11
-
-
34547557698
-
Correctors of protein trafficking defects identified by a novel high-throughput screening assay
-
Carlile G.W., Robert R., Zhang D., Teske K.A., Luo Y., Hanrahan J.W., Thomas D.Y. Correctors of protein trafficking defects identified by a novel high-throughput screening assay. ChemBioChem 2007, 8:1012-1020.
-
(2007)
ChemBioChem
, vol.8
, pp. 1012-1020
-
-
Carlile, G.W.1
Robert, R.2
Zhang, D.3
Teske, K.A.4
Luo, Y.5
Hanrahan, J.W.6
Thomas, D.Y.7
-
12
-
-
0035354585
-
Changes in biomolecular conformation seen by small angle X-ray scattering
-
Doniach S. Changes in biomolecular conformation seen by small angle X-ray scattering. Chem. Rev. 2001, 101:1763-1778.
-
(2001)
Chem. Rev.
, vol.101
, pp. 1763-1778
-
-
Doniach, S.1
-
13
-
-
84908227883
-
Low-resolution structures of OmpA·DDM protein-detergent complexes
-
Døvling Kaspersen J., Moestrup Jessen C., Stougaard Vad B., Skipper Sørensen E., Kleiner Andersen K., Glasius M., Pinto Oliveira C.L., Otzen D.E., Pedersen J.S. Low-resolution structures of OmpA·DDM protein-detergent complexes. ChemBioChem 2014, 15:2113-2124.
-
(2014)
ChemBioChem
, vol.15
, pp. 2113-2124
-
-
Døvling Kaspersen, J.1
Moestrup Jessen, C.2
Stougaard Vad, B.3
Skipper Sørensen, E.4
Kleiner Andersen, K.5
Glasius, M.6
Pinto Oliveira, C.L.7
Otzen, D.E.8
Pedersen, J.S.9
-
14
-
-
65249147217
-
Cooperative assembly and misfolding of CFTR domains in vivo
-
Du K., Lukacs G.L. Cooperative assembly and misfolding of CFTR domains in vivo. Mol. Biol. Cell 2009, 20:1903-1915.
-
(2009)
Mol. Biol. Cell
, vol.20
, pp. 1903-1915
-
-
Du, K.1
Lukacs, G.L.2
-
15
-
-
11444266284
-
The DeltaF508 cystic fibrosis mutation impairs domain-domain interactions and arrests post-translational folding of CFTR
-
Du K., Sharma M., Lukacs G.L. The DeltaF508 cystic fibrosis mutation impairs domain-domain interactions and arrests post-translational folding of CFTR. Nat. Struct. Mol. Biol. 2005, 12:17-25.
-
(2005)
Nat. Struct. Mol. Biol.
, vol.12
, pp. 17-25
-
-
Du, K.1
Sharma, M.2
Lukacs, G.L.3
-
16
-
-
80052321691
-
CFTR three-dimensional structure
-
Ford R.C., Birtley J., Rosenberg M.F., Zhang L. CFTR three-dimensional structure. Methods Mol. Biol. 2011, 741:329-346.
-
(2011)
Methods Mol. Biol.
, vol.741
, pp. 329-346
-
-
Ford, R.C.1
Birtley, J.2
Rosenberg, M.F.3
Zhang, L.4
-
17
-
-
6344243431
-
Interpretation of small-angle scattering data of inhomogeneous ellipsoids
-
Fritz G., Bergmann A. Interpretation of small-angle scattering data of inhomogeneous ellipsoids. J. Appl. Cryst. 2004, 37:815-822.
-
(2004)
J. Appl. Cryst.
, vol.37
, pp. 815-822
-
-
Fritz, G.1
Bergmann, A.2
-
19
-
-
79959715752
-
Small-angle X-ray scattering study of the ATP modulation of the structural features of the nucleotide binding domains of the CFTR in solution
-
Galeno L., Galfrè E., Moran O. Small-angle X-ray scattering study of the ATP modulation of the structural features of the nucleotide binding domains of the CFTR in solution. Eur. Biophys. J. 2011, 40:811-824.
-
(2011)
Eur. Biophys. J.
, vol.40
, pp. 811-824
-
-
Galeno, L.1
Galfrè, E.2
Moran, O.3
-
20
-
-
84867891696
-
A potentiator induces conformational changes on the recombinant CFTR nucleotide binding domains in solution
-
Galfrè E., Galeno L., Moran O. A potentiator induces conformational changes on the recombinant CFTR nucleotide binding domains in solution. Cell. Mol. Life Sci. 2012, 69:3701-3713.
-
(2012)
Cell. Mol. Life Sci.
, vol.69
, pp. 3701-3713
-
-
Galfrè, E.1
Galeno, L.2
Moran, O.3
-
21
-
-
84885670457
-
Managing the underlying cause of cystic fibrosis: a future role for potentiators and correctors
-
Galietta L.J.V. Managing the underlying cause of cystic fibrosis: a future role for potentiators and correctors. Paediatr. Drugs 2013, 15:393-402.
-
(2013)
Paediatr. Drugs
, vol.15
, pp. 393-402
-
-
Galietta, L.J.V.1
-
22
-
-
15944377776
-
Identification of CFTR activators and inhibitors: chance or design?
-
Galietta L.J.V., Moran O. Identification of CFTR activators and inhibitors: chance or design?. Curr. Opin. Pharmacol. 2004, 4:497-503.
-
(2004)
Curr. Opin. Pharmacol.
, vol.4
, pp. 497-503
-
-
Galietta, L.J.V.1
Moran, O.2
-
23
-
-
0000897622
-
A new method for the evaluation of small-angle scattering data
-
Glatter O. A new method for the evaluation of small-angle scattering data. J. Appl. Crystallogr. 1977, 10:415-421.
-
(1977)
J. Appl. Crystallogr.
, vol.10
, pp. 415-421
-
-
Glatter, O.1
-
24
-
-
0001757015
-
The interpretation of real-space information from small-angle scattering experiments
-
Glatter O. The interpretation of real-space information from small-angle scattering experiments. J. Appl. Crystallogr. 1979, 12:166-175.
-
(1979)
J. Appl. Crystallogr.
, vol.12
, pp. 166-175
-
-
Glatter, O.1
-
25
-
-
0001372919
-
Interpretation
-
Academic Press, London, O. Glatter, O. Kratky (Eds.)
-
Glatter O. Interpretation. Small Angle X-ray Scattering 1982, 167-196. Academic Press, London. O. Glatter, O. Kratky (Eds.).
-
(1982)
Small Angle X-ray Scattering
, pp. 167-196
-
-
Glatter, O.1
-
26
-
-
84873436500
-
Correctors of δF508 CFTR restore global conformational maturation without thermally stabilizing the mutant protein
-
He L., Kota P., Aleksandrov A.A., Cui L., Jensen T., Dokholyan N.V., Riordan J.R. Correctors of δF508 CFTR restore global conformational maturation without thermally stabilizing the mutant protein. FASEB J. 2013, 27:536-545.
-
(2013)
FASEB J.
, vol.27
, pp. 536-545
-
-
He, L.1
Kota, P.2
Aleksandrov, A.A.3
Cui, L.4
Jensen, T.5
Dokholyan, N.V.6
Riordan, J.R.7
-
27
-
-
0028858161
-
Multiple proteolytic systems, including the proteasome, contribute to CFTR processing
-
Jensen T.J., Loo M.A., Pind S., Williams D.B., Goldberg A.L., Riordan J.R. Multiple proteolytic systems, including the proteasome, contribute to CFTR processing. Cell 1995, 83:129-135.
-
(1995)
Cell
, vol.83
, pp. 129-135
-
-
Jensen, T.J.1
Loo, M.A.2
Pind, S.3
Williams, D.B.4
Goldberg, A.L.5
Riordan, J.R.6
-
28
-
-
0035450034
-
A sucrose solution application to the study of model biological membranes
-
Kiselev M., Lesieur P., Kisselev A., Lombardo D., Killany M., Lesieur S., Ollivon M. A sucrose solution application to the study of model biological membranes. Nucl. Instrum. Methods Phys. Res. A 2001, 470:409-416.
-
(2001)
Nucl. Instrum. Methods Phys. Res. A
, vol.470
, pp. 409-416
-
-
Kiselev, M.1
Lesieur, P.2
Kisselev, A.3
Lombardo, D.4
Killany, M.5
Lesieur, S.6
Ollivon, M.7
-
29
-
-
0345169163
-
Small-angle scattering: a view on the properties, structures and structural changes of biological macromolecules in solution
-
Koch M.H., Vachette P., Svergun D.I. Small-angle scattering: a view on the properties, structures and structural changes of biological macromolecules in solution. Q. Rev. Biophys. 2003, 36:147-227.
-
(2003)
Q. Rev. Biophys.
, vol.36
, pp. 147-227
-
-
Koch, M.H.1
Vachette, P.2
Svergun, D.I.3
-
30
-
-
84887110575
-
Ab initio and all-atom modeling of detergent organization around Aquaporin-based on SAXS data
-
Koutsioubas A., Berthaud A., Mangenot S., Pérez J. Ab initio and all-atom modeling of detergent organization around Aquaporin-based on SAXS data. J. Phys. Chem. B 2013, 117:13588-13594.
-
(2013)
J. Phys. Chem. B
, vol.117
, pp. 13588-13594
-
-
Koutsioubas, A.1
Berthaud, A.2
Mangenot, S.3
Pérez, J.4
-
31
-
-
10744230777
-
Structure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulator
-
Lewis H.A., Buchanan S.G., Burley S.K., Conners K., Dickey M., Dorwart M., Fowler R., Gao X., Guggino W.B., Hendrickson W.A., et al. Structure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulator. EMBO J. 2004, 23:282-293.
-
(2004)
EMBO J.
, vol.23
, pp. 282-293
-
-
Lewis, H.A.1
Buchanan, S.G.2
Burley, S.K.3
Conners, K.4
Dickey, M.5
Dorwart, M.6
Fowler, R.7
Gao, X.8
Guggino, W.B.9
Hendrickson, W.A.10
-
32
-
-
77449160593
-
Structure and dynamics of NBD1 from CFTR characterized using crystallography and hydrogen/deuterium exchange mass spectrometry
-
Lewis H.A., Wang C., Zhao X., Hamuro Y., Conners K., Kearins M.C., Lu F., Sauder J.M., Molnar K.S., Coales S.J., et al. Structure and dynamics of NBD1 from CFTR characterized using crystallography and hydrogen/deuterium exchange mass spectrometry. J. Mol. Biol. 2010, 396:406-430.
-
(2010)
J. Mol. Biol.
, vol.396
, pp. 406-430
-
-
Lewis, H.A.1
Wang, C.2
Zhao, X.3
Hamuro, Y.4
Conners, K.5
Kearins, M.C.6
Lu, F.7
Sauder, J.M.8
Molnar, K.S.9
Coales, S.J.10
-
33
-
-
19944432524
-
Impact of the DeltaF508 mutation in first nucleotide-binding domain of human cystic fibrosis transmembrane conductance regulator on domain folding and structure
-
Lewis H.A., Zhao X., Wang C., Sauder J.M., Rooney I., Noland B.W., Lorimer D., Kearins M.C., Conners K., Condon B., et al. Impact of the DeltaF508 mutation in first nucleotide-binding domain of human cystic fibrosis transmembrane conductance regulator on domain folding and structure. J. Biol. Chem. 2005, 280:1346-1353.
-
(2005)
J. Biol. Chem.
, vol.280
, pp. 1346-1353
-
-
Lewis, H.A.1
Zhao, X.2
Wang, C.3
Sauder, J.M.4
Rooney, I.5
Noland, B.W.6
Lorimer, D.7
Kearins, M.C.8
Conners, K.9
Condon, B.10
-
34
-
-
84856629737
-
CFTR: folding, misfolding and correcting the δF508 conformational defect
-
Lukacs G.L., Verkman A.S. CFTR: folding, misfolding and correcting the δF508 conformational defect. Trends Mol. Med. 2012, 18:81-91.
-
(2012)
Trends Mol. Med.
, vol.18
, pp. 81-91
-
-
Lukacs, G.L.1
Verkman, A.S.2
-
35
-
-
0018778063
-
Structure of serum low-density lipoprotein. I. A solution X-ray scattering study of a hyperlipidemic monkey low-density lipoprotein
-
Luzzati V., Tardieu A., Aggerbeck L.P. Structure of serum low-density lipoprotein. I. A solution X-ray scattering study of a hyperlipidemic monkey low-density lipoprotein. J. Mol. Biol. 1979, 131:435-473.
-
(1979)
J. Mol. Biol.
, vol.131
, pp. 435-473
-
-
Luzzati, V.1
Tardieu, A.2
Aggerbeck, L.P.3
-
36
-
-
0017234004
-
Structure of human serum lipoproteins in solution. I. Theory and techniques of an X-ray scattering approach using solvents of variable density
-
Luzzati V., Tardieu A., Mateu L., Stuhrmann H. Structure of human serum lipoproteins in solution. I. Theory and techniques of an X-ray scattering approach using solvents of variable density. J. Mol. Biol. 1976, 101:115-127.
-
(1976)
J. Mol. Biol.
, vol.101
, pp. 115-127
-
-
Luzzati, V.1
Tardieu, A.2
Mateu, L.3
Stuhrmann, H.4
-
37
-
-
0037020097
-
High-affinity activators of cystic fibrosis transmembrane conductance regulator (CFTR) chloride conductance identified by high-throughput screening
-
Ma T., Vetrivel L., Yang H., Pedemonte N., Zegarra-Moran O., Galietta L.J.V., Verkman A.S. High-affinity activators of cystic fibrosis transmembrane conductance regulator (CFTR) chloride conductance identified by high-throughput screening. J. Biol. Chem. 2002, 277:37235-37241.
-
(2002)
J. Biol. Chem.
, vol.277
, pp. 37235-37241
-
-
Ma, T.1
Vetrivel, L.2
Yang, H.3
Pedemonte, N.4
Zegarra-Moran, O.5
Galietta, L.J.V.6
Verkman, A.S.7
-
38
-
-
84873717521
-
A SAXS-based ensemble model of the native and phosphorylated regulatory domain of the CFTR
-
Marasini C., Galeno L., Moran O. A SAXS-based ensemble model of the native and phosphorylated regulatory domain of the CFTR. Cell. Mol. Life Sci. 2013, 70:923-933.
-
(2013)
Cell. Mol. Life Sci.
, vol.70
, pp. 923-933
-
-
Marasini, C.1
Galeno, L.2
Moran, O.3
-
39
-
-
77956190770
-
Structural characterization of proteins and complexes using small-angle X-ray solution scattering
-
Mertens H.D.T., Svergun D.I. Structural characterization of proteins and complexes using small-angle X-ray solution scattering. J. Struct. Biol. 2010, 172:128-141.
-
(2010)
J. Struct. Biol.
, vol.172
, pp. 128-141
-
-
Mertens, H.D.T.1
Svergun, D.I.2
-
40
-
-
57649178312
-
Three-dimensional reconstruction of human cystic fibrosis transmembrane conductance regulator chloride channel revealed an ellipsoidal structure with orifices beneath the putative transmembrane domain
-
Mio K., Ogura T., Mio M., Shimizu H., Hwang T., Sato C., Sohma Y. Three-dimensional reconstruction of human cystic fibrosis transmembrane conductance regulator chloride channel revealed an ellipsoidal structure with orifices beneath the putative transmembrane domain. J. Biol. Chem. 2008, 283:30300-30310.
-
(2008)
J. Biol. Chem.
, vol.283
, pp. 30300-30310
-
-
Mio, K.1
Ogura, T.2
Mio, M.3
Shimizu, H.4
Hwang, T.5
Sato, C.6
Sohma, Y.7
-
41
-
-
84901610851
-
On the structural organization of the intracellular domains of CFTR
-
Moran O. On the structural organization of the intracellular domains of CFTR. Int. J. Biochem. Cell Biol. 2014, 52C:7-14.
-
(2014)
Int. J. Biochem. Cell Biol.
, vol.52C
, pp. 7-14
-
-
Moran, O.1
-
42
-
-
70349847830
-
Molecular models of the open and closed states of the whole human CFTR protein
-
Mornon J., Lehn P., Callebaut I. Molecular models of the open and closed states of the whole human CFTR protein. Cell. Mol. Life Sci. 2009, 66:3469-3486.
-
(2009)
Cell. Mol. Life Sci.
, vol.66
, pp. 3469-3486
-
-
Mornon, J.1
Lehn, P.2
Callebaut, I.3
-
43
-
-
84928493859
-
Full-open and closed CFTR channels, with lateral tunnels from the cytoplasm and an alternative position of the F508 region, as revealed by molecular dynamics
-
Mornon J.-P., Hoffmann B., Jonic S., Lehn P., Callebaut I. Full-open and closed CFTR channels, with lateral tunnels from the cytoplasm and an alternative position of the F508 region, as revealed by molecular dynamics. Cell. Mol. Life Sci. 2015, 72:1377-1403.
-
(2015)
Cell. Mol. Life Sci.
, vol.72
, pp. 1377-1403
-
-
Mornon, J.-P.1
Hoffmann, B.2
Jonic, S.3
Lehn, P.4
Callebaut, I.5
-
44
-
-
0029063514
-
Purification and characterization of recombinant cystic fibrosis transmembrane conductance regulator from Chinese hamster ovary and insect cells
-
O'Riordan C.R., Erickson A., Bear C., Li C., Manavalan P., Wang K.X., Marshall J., Scheule R.K., McPherson J.M., Cheng S.H. Purification and characterization of recombinant cystic fibrosis transmembrane conductance regulator from Chinese hamster ovary and insect cells. J. Biol. Chem. 1995, 270:17033-17043.
-
(1995)
J. Biol. Chem.
, vol.270
, pp. 17033-17043
-
-
O'Riordan, C.R.1
Erickson, A.2
Bear, C.3
Li, C.4
Manavalan, P.5
Wang, K.X.6
Marshall, J.7
Scheule, R.K.8
McPherson, J.M.9
Cheng, S.H.10
-
45
-
-
84863767664
-
Expression and purification of the cystic fibrosis transmembrane conductance regulator protein in Saccharomyces cerevisiae
-
O'Ryan L., Rimington T., Cant N., Ford R.C. Expression and purification of the cystic fibrosis transmembrane conductance regulator protein in Saccharomyces cerevisiae. J. Vis. Exp. 2012, 61:1-7.
-
(2012)
J. Vis. Exp.
, vol.61
, pp. 1-7
-
-
O'Ryan, L.1
Rimington, T.2
Cant, N.3
Ford, R.C.4
-
46
-
-
84884967922
-
Discovery of novel potent δF508-CFTR correctors that target the nucleotide binding domain
-
Odolczyk N., Fritsch J., Norez C., Servel N., da Cunha M.F., Bitam S., Kupniewska A., Wiszniewski L., Colas J., Tarnowski K., et al. Discovery of novel potent δF508-CFTR correctors that target the nucleotide binding domain. EMBO Mol. Med. 2013, 5:1484-1501.
-
(2013)
EMBO Mol. Med.
, vol.5
, pp. 1484-1501
-
-
Odolczyk, N.1
Fritsch, J.2
Norez, C.3
Servel, N.4
da Cunha, M.F.5
Bitam, S.6
Kupniewska, A.7
Wiszniewski, L.8
Colas, J.9
Tarnowski, K.10
-
47
-
-
84877310319
-
Dependence of micelle size and shape on detergent alkyl chain length and head group
-
Oliver R.C., Lipfert J., Fox D.A., Lo R.H., Doniach S., Columbus L. Dependence of micelle size and shape on detergent alkyl chain length and head group. PLoS ONE 2013, 8.
-
(2013)
PLoS ONE
, vol.8
-
-
Oliver, R.C.1
Lipfert, J.2
Fox, D.A.3
Lo, R.H.4
Doniach, S.5
Columbus, L.6
-
48
-
-
35748982423
-
Analysis of X-ray and neutron scattering from biomacromolecular solutions
-
Petoukhov M.V., Svergun D.I. Analysis of X-ray and neutron scattering from biomacromolecular solutions. Curr. Opin. Struct. Biol. 2007, 17:562-571.
-
(2007)
Curr. Opin. Struct. Biol.
, vol.17
, pp. 562-571
-
-
Petoukhov, M.V.1
Svergun, D.I.2
-
49
-
-
84859782518
-
New developments in the ATSAS program package for small-angle scattering data analysis
-
Petoukhov M.V., Franke D., Shkumatov A.V., Tria G., Kikhney A.G., Gajda M., Gorba C., Mertens H.D.T., Konarev P.V., Svergun D.I. New developments in the ATSAS program package for small-angle scattering data analysis. J. Appl. Crystallogr. 2012, 45:342-350.
-
(2012)
J. Appl. Crystallogr.
, vol.45
, pp. 342-350
-
-
Petoukhov, M.V.1
Franke, D.2
Shkumatov, A.V.3
Tria, G.4
Kikhney, A.G.5
Gajda, M.6
Gorba, C.7
Mertens, H.D.T.8
Konarev, P.V.9
Svergun, D.I.10
-
50
-
-
84900385883
-
Purification of the cystic fibrosis transmembrane conductance regulator protein expressed in Saccharomyces cerevisiae
-
Pollock N., Cant N., Rimington T., Ford R.C. Purification of the cystic fibrosis transmembrane conductance regulator protein expressed in Saccharomyces cerevisiae. JoVE 2014, 52:15-25.
-
(2014)
JoVE
, vol.52
, pp. 15-25
-
-
Pollock, N.1
Cant, N.2
Rimington, T.3
Ford, R.C.4
-
51
-
-
84947277557
-
Characterisation of the salmon cystic fibrosis transmembrane conductance regulator protein for structural studies
-
Pollock N.L., Moran O., Baroni D., Zegarra-Moran O., Ford R.C. Characterisation of the salmon cystic fibrosis transmembrane conductance regulator protein for structural studies. AIMS Mol. Sci. 2014, 1:141-161.
-
(2014)
AIMS Mol. Sci.
, vol.1
, pp. 141-161
-
-
Pollock, N.L.1
Moran, O.2
Baroni, D.3
Zegarra-Moran, O.4
Ford, R.C.5
-
52
-
-
0030669141
-
A novel procedure for the efficient purification of the cystic fibrosis transmembrane conductance regulator (CFTR)
-
Ramjeesingh M., Li C., Garami E., Huan L.J., Hewryk M., Wang Y., Galley K., Bear C.E. A novel procedure for the efficient purification of the cystic fibrosis transmembrane conductance regulator (CFTR). Biochem. J. 1997, 327(Pt 1):17-21.
-
(1997)
Biochem. J.
, vol.327
, pp. 17-21
-
-
Ramjeesingh, M.1
Li, C.2
Garami, E.3
Huan, L.J.4
Hewryk, M.5
Wang, Y.6
Galley, K.7
Bear, C.E.8
-
53
-
-
80455162465
-
A CFTR potentiator in patients with cystic fibrosis and the G551D mutation
-
Ramsey B.W., Davies J., McElvaney N.G., Tullis E., Bell S.C., Dřevínek P., Griese M., McKone E.F., Wainwright C.E., Konstan M.W., et al. A CFTR potentiator in patients with cystic fibrosis and the G551D mutation. N. Engl. J. Med. 2011, 365:1663-1672.
-
(2011)
N. Engl. J. Med.
, vol.365
, pp. 1663-1672
-
-
Ramsey, B.W.1
Davies, J.2
McElvaney, N.G.3
Tullis, E.4
Bell, S.C.5
Dřevínek, P.6
Griese, M.7
McKone, E.F.8
Wainwright, C.E.9
Konstan, M.W.10
-
54
-
-
84884773595
-
VX-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1
-
Ren H.Y., Grove D.E., De La Rosa O., Houck S.A., Sopha P., Van Goor F., Hoffman B.J., Cyr D.M. VX-809 corrects folding defects in cystic fibrosis transmembrane conductance regulator protein through action on membrane-spanning domain 1. Mol. Biol. Cell 2013, 24:3016-3024.
-
(2013)
Mol. Biol. Cell
, vol.24
, pp. 3016-3024
-
-
Ren, H.Y.1
Grove, D.E.2
De La Rosa, O.3
Houck, S.A.4
Sopha, P.5
Van Goor, F.6
Hoffman, B.J.7
Cyr, D.M.8
-
55
-
-
0024424270
-
Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA
-
Riordan J.R., Rommens J.M., Kerem B., Alon N., Rozmahel R., Grzelczak Z., Zielenski J., Lok S., Plavsic N., Chou J.L., et al. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 1989, 245:1066-1073.
-
(1989)
Science
, vol.245
, pp. 1066-1073
-
-
Riordan, J.R.1
Rommens, J.M.2
Kerem, B.3
Alon, N.4
Rozmahel, R.5
Grzelczak, Z.6
Zielenski, J.7
Lok, S.8
Plavsic, N.9
Chou, J.L.10
-
56
-
-
4544378176
-
Purification and crystallization of the cystic fibrosis transmembrane conductance regulator (CFTR)
-
Rosenberg M.F., Kamis A.B., Aleksandrov L.A., Ford R.C., Riordan J.R. Purification and crystallization of the cystic fibrosis transmembrane conductance regulator (CFTR). J. Biol. Chem. 2004, 279:39051-39057.
-
(2004)
J. Biol. Chem.
, vol.279
, pp. 39051-39057
-
-
Rosenberg, M.F.1
Kamis, A.B.2
Aleksandrov, L.A.3
Ford, R.C.4
Riordan, J.R.5
-
57
-
-
82755176172
-
The cystic fibrosis transmembrane conductance regulator (CFTR): three-dimensional structure and localization of a channel gate
-
Rosenberg M.F., O'Ryan L.P., Hughes G., Zhao Z., Aleksandrov L.A., Riordan J.R., Ford R.C. The cystic fibrosis transmembrane conductance regulator (CFTR): three-dimensional structure and localization of a channel gate. J. Biol. Chem. 2011, 286:42647-42654.
-
(2011)
J. Biol. Chem.
, vol.286
, pp. 42647-42654
-
-
Rosenberg, M.F.1
O'Ryan, L.P.2
Hughes, G.3
Zhao, Z.4
Aleksandrov, L.A.5
Riordan, J.R.6
Ford, R.C.7
-
58
-
-
0017130118
-
Shape and size of bovine rhodopsin: a small-angle X-ray scattering study of a rhodopsin-detergent complex
-
Sardet C., Tardieu A., Luzzati V. Shape and size of bovine rhodopsin: a small-angle X-ray scattering study of a rhodopsin-detergent complex. J. Mol. Biol. 1976, 105:383-407.
-
(1976)
J. Mol. Biol.
, vol.105
, pp. 383-407
-
-
Sardet, C.1
Tardieu, A.2
Luzzati, V.3
-
59
-
-
2742606422
-
Contrast variation
-
Academic Press, London, O. Glatter, O. Kratky (Eds.)
-
Stuhrmann H.B. Contrast variation. Small Angle X-ray Scattering 1982, 197-213. Academic Press, London. O. Glatter, O. Kratky (Eds.).
-
(1982)
Small Angle X-ray Scattering
, pp. 197-213
-
-
Stuhrmann, H.B.1
-
60
-
-
0026910457
-
Determination of the regularization parameter in indirect-transform methods using perceptual criteria
-
Svergun D.I. Determination of the regularization parameter in indirect-transform methods using perceptual criteria. J. Appl. Crystallogr. 1992, 25:495-503.
-
(1992)
J. Appl. Crystallogr.
, vol.25
, pp. 495-503
-
-
Svergun, D.I.1
-
61
-
-
0242571958
-
Small-angle scattering studies of biological macromolecules in solution
-
Svergun D.I., Koch M.H. Small-angle scattering studies of biological macromolecules in solution. Rep. Prog. Phys. 2003, 66:1735-1782.
-
(2003)
Rep. Prog. Phys.
, vol.66
, pp. 1735-1782
-
-
Svergun, D.I.1
Koch, M.H.2
-
62
-
-
0017307778
-
Structure of human serum lipoproteins in solution. II. Small-angle X-ray scattering study of HDL3 and LDL
-
Tardieu A., Mateu L., Sardet C., Weiss B., Luzzati V., Aggerbeck L., Scanu A.M. Structure of human serum lipoproteins in solution. II. Small-angle X-ray scattering study of HDL3 and LDL. J. Mol. Biol. 1976, 105:459-460.
-
(1976)
J. Mol. Biol.
, vol.105
, pp. 459-460
-
-
Tardieu, A.1
Mateu, L.2
Sardet, C.3
Weiss, B.4
Luzzati, V.5
Aggerbeck, L.6
Scanu, A.M.7
-
63
-
-
11444265307
-
Side chain and backbone contributions of Phe508 to CFTR folding
-
Thibodeau P.H., Brautigam C.A., Machius M., Thomas P.J. Side chain and backbone contributions of Phe508 to CFTR folding. Nat. Struct. Mol. Biol. 2005, 12:10-16.
-
(2005)
Nat. Struct. Mol. Biol.
, vol.12
, pp. 10-16
-
-
Thibodeau, P.H.1
Brautigam, C.A.2
Machius, M.3
Thomas, P.J.4
-
64
-
-
81755163563
-
Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809
-
Van Goor F., Hadida S., Grootenhuis P.D.J., Burton B., Stack J.H., Straley K.S., Decker C.J., Miller M., McCartney J., Olson E.R., et al. Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809. Proc. Natl. Acad. Sci. U.S.A. 2011, 108:18843-18848.
-
(2011)
Proc. Natl. Acad. Sci. U.S.A.
, vol.108
, pp. 18843-18848
-
-
Van Goor, F.1
Hadida, S.2
Grootenhuis, P.D.J.3
Burton, B.4
Stack, J.H.5
Straley, K.S.6
Decker, C.J.7
Miller, M.8
McCartney, J.9
Olson, E.R.10
-
65
-
-
33744831154
-
Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules
-
Van Goor F., Straley K.S., Cao D., González J., Hadida S., Hazlewood A., Joubran J., Knapp T., Makings L.R., Miller M., et al. Rescue of DeltaF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules. Am. J. Physiol. Lung Cell. Mol. Physiol. 2006, 290:L1117-L1130.
-
(2006)
Am. J. Physiol. Lung Cell. Mol. Physiol.
, vol.290
, pp. L1117-L1130
-
-
Van Goor, F.1
Straley, K.S.2
Cao, D.3
González, J.4
Hadida, S.5
Hazlewood, A.6
Joubran, J.7
Knapp, T.8
Makings, L.R.9
Miller, M.10
-
66
-
-
84884239437
-
Detection of phospho-sites generated by protein kinase CK2 in CFTR: mechanistic aspects of Thr1471 phosphorylation
-
Venerando A., Franchin C., Cant N., Cozza G., Pagano M.A., Tosoni K., Al-Zahrani A., Arrigoni G., Ford R.C., Mehta A., et al. Detection of phospho-sites generated by protein kinase CK2 in CFTR: mechanistic aspects of Thr1471 phosphorylation. PLoS ONE 2013, 8.
-
(2013)
PLoS ONE
, vol.8
-
-
Venerando, A.1
Franchin, C.2
Cant, N.3
Cozza, G.4
Pagano, M.A.5
Tosoni, K.6
Al-Zahrani, A.7
Arrigoni, G.8
Ford, R.C.9
Mehta, A.10
-
67
-
-
84904174102
-
Membrane protein stability can be compromised by detergent interactions with the extramembranous soluble domains
-
Yang Z., Wang C., Zhou Q., An J., Hildebrandt E., Aleksandrov L.A., Kappes J.C., DeLucas L.J., Riordan J.R., Urbatsch I.L., et al. Membrane protein stability can be compromised by detergent interactions with the extramembranous soluble domains. Protein Sci. 2014, 23:769-789.
-
(2014)
Protein Sci.
, vol.23
, pp. 769-789
-
-
Yang, Z.1
Wang, C.2
Zhou, Q.3
An, J.4
Hildebrandt, E.5
Aleksandrov, L.A.6
Kappes, J.C.7
DeLucas, L.J.8
Riordan, J.R.9
Urbatsch, I.L.10
-
68
-
-
33746675669
-
Sequential quality-control checkpoints triage misfolded cystic fibrosis transmembrane conductance regulator
-
Younger J.M., Chen L., Ren H.Y., Rosser M.F., Turnbull E.L., Fan C.Y., Patterson C., Cyr D.M. Sequential quality-control checkpoints triage misfolded cystic fibrosis transmembrane conductance regulator. Cell 2006, 126:571-582.
-
(2006)
Cell
, vol.126
, pp. 571-582
-
-
Younger, J.M.1
Chen, L.2
Ren, H.Y.3
Rosser, M.F.4
Turnbull, E.L.5
Fan, C.Y.6
Patterson, C.7
Cyr, D.M.8
-
69
-
-
78649761241
-
Domain location within the cystic fibrosis transmembrane conductance regulator protein investigated by electron microscopy and gold labelling
-
Zhang L., Aleksandrov L.A., Riordan J.R., Ford R.C. Domain location within the cystic fibrosis transmembrane conductance regulator protein investigated by electron microscopy and gold labelling. Biochim. Biophys. Acta 2011, 1808:399-404.
-
(2011)
Biochim. Biophys. Acta
, vol.1808
, pp. 399-404
-
-
Zhang, L.1
Aleksandrov, L.A.2
Riordan, J.R.3
Ford, R.C.4
|