Localizing a gate in CFTR

Proceedings of the National Academy of Sciences of the United States of America

Volumn 112, Issue 8, 2015, Pages 2461-2466

  Gao, Xiaolong ^a,b   Hwang, Tzyh Chang ^a,b,c  

^a UNIVERSITY OF MISSOURI   (United States)

^b UNIVERSITY OF MISSOURI   (United States)

^c University of Missouri   (United States)

Author keywords

Abc transporters; Anion channels; Cystic fibrosis; Gating

Indexed keywords

ABC TRANSPORTER; AMINO ACID; CHLORIDE ION; CYSTEINE; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; CYANIC ACID DERIVATIVE; GOLD; GOLD CYANIDE; MUTANT PROTEIN; THIOL DERIVATIVE;

ANIMAL CELL; ARTICLE; CELL MEMBRANE POTENTIAL; CHEMICAL STRUCTURE; CHO CELL LINE; CONFORMATIONAL TRANSITION; CONTROLLED STUDY; CRYSTAL STRUCTURE; DEPHOSPHORYLATION; DNA SEQUENCE; FEMALE; INSIDE OUT PATCH CLAMP; ION TRANSPORT; MUTATION; NONHUMAN; PHYLOGENY; PRIORITY JOURNAL; PROTEIN ENGINEERING; PROTEIN EXPRESSION; PROTEIN LOCALIZATION; REACTION TIME; STEADY STATE; VESTIBULE; WHOLE CELL PATCH CLAMP; ANIMAL; CHANNEL GATING; CHEMISTRY; CRICETULUS; HAMSTER; KINETICS; METABOLISM;

ANIMALS; CHO CELLS; CRICETINAE; CRICETULUS; CYANATES; CYSTEINE; CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR; GOLD; ION CHANNEL GATING; KINETICS; MUTANT PROTEINS; SULFHYDRYL COMPOUNDS;

EID: 84923668367     PISSN: 00278424     EISSN: 10916490     Source Type: Journal    
DOI: 10.1073/pnas.1420676112     Document Type: Article

References (46)

1. Ashcroft F, Gadsby D, Miller C (2009) Introduction. The blurred boundary between channels and transporters. Philos Trans R Soc Lond B Biol Sci 364(1514):145-147.
2. Jayaram H, Accardi A, Wu F, Williams C, Miller C (2008) Ion permeation through a Clselective channel designed from a CLC Cl-/H+ exchanger. Proc Natl Acad Sci USA 105(32):11194-11199.
3. Rees DC, Johnson E, Lewinson O (2009) ABC transporters: The power to change. Nat Rev Mol Cell Biol 10(3):218-227.
4. Jardetzky O (1966) Simple allosteric model for membrane pumps. Nature 211(5052): 969-970.
5. Dawson RJ, Locher KP (2006) Structure of a bacterial multidrug ABC transporter. Nature 443(7108):180-185.
6. Ward A, Reyes CL, Yu J, Roth CB, Chang G (2007) Flexibility in the ABC transporter MsbA: Alternating access with a twist. Proc Natl Acad Sci USA 104(48):19005-19010.
7. Aller SG, et al. (2009) Structure of P-glycoprotein reveals a molecular basis for polyspecific drug binding. Science 323(5922):1718-1722.
8. Hohl M, Briand C, Grütter MG, Seeger MA (2012) Crystal structure of a heterodimeric ABC transporter in its inward-facing conformation. Nat Struct Mol Biol 19(4):395-402.
9. Chen TY, Hwang TC (2008) CLC-0 and CFTR: Chloride channels evolved from transporters. Physiol Rev 88(2):351-387.
10. Dean M, Annilo T (2005) Evolution of the ATP-binding cassette (ABC) transporter superfamily in vertebrates. Annu Rev Genomics Hum Genet 6:123-142.
11. Riordan JR, et al. (1989) Identification of the cystic fibrosis gene: Cloning and characterization of complementary DNA. Science 245(4922):1066-1073.
12. Hwang TC, Sheppard DN (2009) Gating of the CFTR Cl- channel by ATP-driven nucleotide-binding domain dimerisation. J Physiol 587(Pt 10):2151-2161.
13. Jih KY, Hwang TC (2012) Nonequilibrium gating of CFTR on an equilibrium theme. Physiology (Bethesda) 27(6):351-361.
14. Gadsby DC, Nairn AC (1999) Control of CFTR channel gating by phosphorylation and nucleotide hydrolysis. Physiol Rev 79(1, Suppl):S77-S107.
15. Bai Y, Li M, Hwang TC (2010) Dual roles of the sixth transmembrane segment of the CFTR chloride channel in gating and permeation. J Gen Physiol 136(3):293-309.
16. Bai Y, Li M, Hwang TC (2011) Structural basis for the channel function of a degraded ABC transporter, CFTR (ABCC7). J Gen Physiol 138(5):495-507.
17. El Hiani Y, Linsdell P (2010) Changes in accessibility of cytoplasmic substances to the pore associated with activation of the cystic fibrosis transmembrane conductance regulator chloride channel. J Biol Chem 285(42):32126-32140.
18. th transmembrane region in the CFTR chloride channel pore based on functional investigation of a cysteine-less CFTR variant. Pflugers Arch 462(4):559-571.
19. Wang W, El Hiani Y, Linsdell P (2011) Alignment of transmembrane regions in the cystic fibrosis transmembrane conductance regulator chloride channel pore. J Gen Physiol 138(2):165-178.
20. Gao X, Bai Y, Hwang TC (2013) Cysteine scanning of CFTR's first transmembrane segment reveals its plausible roles in gating and permeation. Biophys J 104(4):786-797.
21. Alexander C, et al. (2009) Cystic fibrosis transmembrane conductance regulator: Using differential reactivity toward channel-permeant and channel-impermeant thiol-reactive probes to test a molecular model for the pore. Biochemistry 48(42):10078-10088.
22. Norimatsu Y, et al. (2012) Cystic fibrosis transmembrane conductance regulator: A molecular model defines the architecture of the anion conduction path and locates a "bottleneck" in the pore. Biochemistry 51(11):2199-2212.
23. McCarty NA, Zhang ZR (2001) Identification of a region of strong discrimination in the pore of CFTR. Am J Physiol Lung Cell Mol Physiol 281(4):L852-L867.
24. Wang W, El Hiani Y, Rubaiy HN, Linsdell P (2014) Relative contribution of different transmembrane segments to the CFTR chloride channel pore. Pflugers Arch 466(3):477-490.
25. El Hiani Y, Linsdell P (2014) Metal bridges illuminate transmembrane domain movements during gating of the cystic fibrosis transmembrane conductance regulator chloride channel. J Biol Chem 289(41):28149-28159.
26. Rahman KS, Cui G, Harvey SC, McCarty NA (2013) Modeling the conformational changes underlying channel opening in CFTR. PLoS ONE 8(9):e74574.
27. Mornon JP, Hoffmann B, Jonic S, Lehn P, Callebaut I (2014) 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, in press.
28. Gadsby DC (2009) Ion channels versus ion pumps: The principal difference, in principle. Nat Rev Mol Cell Biol 10(5):344-352.
29. Smith SS, Steinle ED, Meyerhoff ME, Dawson DC (1999) Cystic fibrosis transmembrane conductance regulator. Physical basis for lyotropic anion selectivity patterns. J Gen Physiol 114(6):799-818.
30. Fatehi M, St Aubin CN, Linsdell P (2007) On the origin of asymmetric interactions between permeant anions and the cystic fibrosis transmembrane conductance regulator chloride channel pore. Biophys J 92(4):1241-1253.
31. Gong X, Burbridge SM, Cowley EA, Linsdell P (2002) Molecular determinants of Au(CN)(2)(-) binding and permeability within the cystic fibrosis transmembrane conductance regulator Cl(-) channel pore. J Physiol 540(Pt 1):39-47.
32. Serrano JR, et al. (2006) CFTR: Ligand exchange between a permeant anion ([Au(CN)2]-) and an engineered cysteine (T338C) blocks the pore. Biophys J 91(5):1737-1748.
33. Liu X, Dawson DC (2011) Cystic fibrosis transmembrane conductance regulator: Temperature-dependent cysteine reactivity suggests different stable conformers of the conduction pathway. Biochemistry 50(47):10311-10317.
34. Bompadre SG, Sohma Y, Li M, Hwang TC (2007) G551D and G1349D, two CF-associated mutations in the signature sequences of CFTR, exhibit distinct gating defects. J Gen Physiol 129(4):285-298.
35. Gregory RJ, et al. (1991) Maturation and function of cystic fibrosis transmembrane conductance regulator variants bearing mutations in putative nucleotide-binding domains 1 and 2. Mol Cell Biol 11(8):3886-3893.
36. Liu X, Alexander C, Serrano J, Borg E, Dawson DC (2006) Variable reactivity of an engineered cysteine at position 338 in cystic fibrosis transmembrane conductance regulator reflects different chemical states of the thiol. J Biol Chem 281(12):8275-8285.
37. Linsdell P (2014) Functional architecture of the CFTR chloride channel. Mol Membr Biol 31(1):1-16.
38. Sheppard DN, Robinson KA (1997) Mechanism of glibenclamide inhibition of cystic fibrosis transmembrane conductance regulator Cl- channels expressed in a murine cell line. J Physiol 503(Pt 2):333-346.
39. Muanprasat C, et al. (2004) Discovery of glycine hydrazide pore-occluding CFTR inhibitors: Mechanism, structure-activity analysis, and in vivo efficacy. J Gen Physiol 124(2):125-137.
40. Norimatsu Y, et al. (2012) Locating a plausible binding site for an open-channel blocker, GlyH-101, in the pore of the cystic fibrosis transmembrane conductance regulator. Mol Pharmacol 82(6):1042-1055.
41. Linsdell P, et al. (1997) Permeability of wild-type and mutant cystic fibrosis transmembrane conductance regulator chloride channels to polyatomic anions. J Gen Physiol 110(4):355-364.
42. Morais-Cabral JH, Zhou Y, MacKinnon R (2001) Energetic optimization of ion conduction rate by the K+ selectivity filter. Nature 414(6859):37-42.
43. Dutzler R, Campbell EB, Cadene M, Chait BT, MacKinnon R (2002) X-ray structure of a ClC chloride channel at 3.0 A reveals the molecular basis of anion selectivity. Nature 415(6869):287-294.
44. Linsdell P, Zheng SX, Hanrahan JW (1998) Non-pore lining amino acid side chains influence anion selectivity of the human CFTR Cl- channel expressed in mammalian cell lines. J Physiol 512(Pt 1):1-16.
45. Linsdell P (2001) Relationship between anion binding and anion permeability revealed by mutagenesis within the cystic fibrosis transmembrane conductance regulator chloride channel pore. J Physiol 531(Pt 1):51-66.
46. Wang W, Linsdell P (2012) Relative movements of transmembrane regions at the outer mouth of the cystic fibrosis transmembrane conductance regulator channel pore during channel gating. J Biol Chem 287(38):32136-32146.