{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,9]],"date-time":"2026-01-09T12:19:19Z","timestamp":1767961159436,"version":"3.49.0"},"reference-count":26,"publisher":"Public Library of Science (PLoS)","issue":"2","license":[{"start":{"date-parts":[[2008,2,29]],"date-time":"2008-02-29T00:00:00Z","timestamp":1204243200000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["www.ploscompbiol.org"],"crossmark-restriction":false},"short-container-title":["PLoS Comput Biol"],"DOI":"10.1371\/journal.pcbi.1000008","type":"journal-article","created":{"date-parts":[[2008,2,28]],"date-time":"2008-02-28T21:37:29Z","timestamp":1204234649000},"page":"e1000008","update-policy":"https:\/\/doi.org\/10.1371\/journal.pcbi.corrections_policy","source":"Crossref","is-referenced-by-count":40,"title":["Diminished Self-Chaperoning Activity of the \u0394F508 Mutant of CFTR Results in Protein Misfolding"],"prefix":"10.1371","volume":"4","author":[{"given":"Adrian W. R.","family":"Serohijos","sequence":"first","affiliation":[]},{"given":"Tam\u00e1s","family":"Heged\u0171s","sequence":"additional","affiliation":[]},{"given":"John R.","family":"Riordan","sequence":"additional","affiliation":[]},{"given":"Nikolay V.","family":"Dokholyan","sequence":"additional","affiliation":[]}],"member":"340","published-online":{"date-parts":[[2008,2,29]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"146","DOI":"10.1183\/09031936.03.00057003","article-title":"New concepts of the pathogenesis of cystic fibrosis lung disease.","volume":"23","author":"RC Boucher","year":"2004","journal-title":"Eur Resp J"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"571","DOI":"10.1172\/JCI0215217","article-title":"Mucus clearance as a primary innate defense mechanism for mammalian airways.","volume":"109","author":"MR Knowles","year":"2002","journal-title":"J Clin Investig"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1097\/ACI.0b013e3280114eef","article-title":"Mucociliary clearance - a critical upper airway host defense mechanism and methods of assessment.","volume":"7","author":"MB Antunes","year":"2007","journal-title":"Curr Opin Allergy Clin Immunol"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"1066","DOI":"10.1126\/science.2475911","article-title":"Identification of the Cystic-Fibrosis Gene - Cloning and Characterization of Complementary-Dna.","volume":"245","author":"JR Riordan","year":"1989","journal-title":"Science"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"1059","DOI":"10.1126\/science.2772657","article-title":"Identification of the Cystic-Fibrosis Gene - Chromosome Walking and Jumping.","volume":"245","author":"JM Rommens","year":"1989","journal-title":"Science"},{"key":"ref6","doi-asserted-by":"crossref","first-page":"575","DOI":"10.1002\/humu.10041","article-title":"Cystic fibrosis: A worldwide analysis of CFTR mutations - Correlation with incidence data and application to screening.","volume":"19","author":"JL Bobadilla","year":"2002","journal-title":"Human Mutation"},{"key":"ref7","doi-asserted-by":"crossref","first-page":"7261","DOI":"10.1074\/jbc.271.13.7261","article-title":"Alteration of the cystic fibrosis transmembrane conductance regulator folding pathway - Effects of the Delta F508 mutation on the thermodynamic stability and folding yield of NBD1.","volume":"271","author":"BH Qu","year":"1996","journal-title":"J Biol Chem"},{"key":"ref8","doi-asserted-by":"crossref","first-page":"15739","DOI":"10.1074\/jbc.272.25.15739","article-title":"Localization and suppression of a kinetic defect in cystic fibrosis transmembrane conductance regulator folding.","volume":"272","author":"BH Qu","year":"1997","journal-title":"J Biol Chem"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1038\/nsmb881","article-title":"Side chain and backbone contributions of Phe508 to CFTR folding.","volume":"12","author":"PH Thibodeau","year":"2005","journal-title":"Nat Struct Mol Biol"},{"key":"ref10","doi-asserted-by":"crossref","first-page":"1346","DOI":"10.1074\/jbc.M410968200","article-title":"Impact of the Delta F508 mutation in first nucleotide-binding domain of human cystic fibrosis transmembrane conductance regulator on domain folding and structure.","volume":"280","author":"HA Lewis","year":"2005","journal-title":"J Biol Chem"},{"key":"ref11","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1038\/nsmb882","article-title":"The F508 cystic fibrosis mutation impairs domain-domain interactions and arrests post-translational folding of CFTR.","volume":"12","author":"K Du","year":"2005","journal-title":"Nat Struct Mol Biol"},{"key":"ref12","doi-asserted-by":"crossref","first-page":"282","DOI":"10.1038\/sj.emboj.7600040","article-title":"Structure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulator.","volume":"23","author":"HA Lewis","year":"2004","journal-title":"EMBO J"},{"key":"ref13","doi-asserted-by":"crossref","first-page":"180","DOI":"10.1038\/nature05155","article-title":"Structure of a bacterial multidrug ABC transporter.","volume":"443","author":"RJP Dawson","year":"2006","journal-title":"Nature"},{"key":"ref14","doi-asserted-by":"crossref","first-page":"1091","DOI":"10.1126\/science.1071142","article-title":"The E-coli BtuCD structure: A framework for ABC transporter architecture and mechanism.","volume":"296","author":"KP Locher","year":"2002","journal-title":"Science"},{"key":"ref15","doi-asserted-by":"crossref","first-page":"35896","DOI":"10.1074\/jbc.M205644200","article-title":"Mutations in the nucleotide binding domain 1 signature motif region rescue processing and functional defects of cystic fibrosis transmembrane conductance regulator Delta F508.","volume":"277","author":"ACV deCarvalho","year":"2002","journal-title":"J Biol Chem"},{"key":"ref16","doi-asserted-by":"crossref","first-page":"335","DOI":"10.1016\/0092-8674(93)90233-G","article-title":"Identification of Revertants for the Cystic-Fibrosis Delta-F508 Mutation Using Ste6-Cftr Chimeras in Yeast.","volume":"73","author":"JL Teem","year":"1993","journal-title":"Cell"},{"key":"ref17","first-page":"63","article-title":"Mutation of R555 in CFTR-Delta F508 enhances function and partially corrects defective processing.","volume":"4","author":"JL Teem","year":"1996","journal-title":"Receptors Channels"},{"key":"ref18","doi-asserted-by":"crossref","first-page":"1457","DOI":"10.1529\/biophysj.106.094805","article-title":"Multiscale Modeling of nucleosome dynamics.","volume":"92","author":"S Sharma","year":"2007","journal-title":"Biophys J"},{"key":"ref19","doi-asserted-by":"crossref","first-page":"577","DOI":"10.1016\/S1359-0278(98)00072-8","article-title":"Discrete molecular dynamics studies of the folding of a protein-like model.","volume":"3","author":"NV Dokholyan","year":"1998","journal-title":"Folding Design"},{"key":"ref20","doi-asserted-by":"crossref","first-page":"450","DOI":"10.1016\/j.tibtech.2005.07.001","article-title":"Simple but predictive protein models.","volume":"23","author":"F Ding","year":"2005","journal-title":"Trends Biotechnol"},{"key":"ref21","doi-asserted-by":"crossref","first-page":"334","DOI":"10.1063\/1.475393","article-title":"On the transition coordinate for protein folding.","volume":"108","author":"R Du","year":"1998","journal-title":"J Chem Phys"},{"key":"ref22","doi-asserted-by":"crossref","first-page":"8942","DOI":"10.1074\/jbc.M009172200","article-title":"Conformational and Temperature-sensitive Stability Defects of the Delta F508 Cystic Fibrosis Transmembrane Conductance Regulator in Post-endoplasmic Reticulum Compartments.","volume":"276","author":"M Sharma","year":"2001","journal-title":"J Biol Chem"},{"key":"ref23","doi-asserted-by":"crossref","first-page":"17891","DOI":"10.1073\/pnas.0608312103","article-title":"Revertant mutants G550E and 4RK rescue cystic fibrosis mutants in the first nucleotide-binding domain of CFTR by different mechanisms.","volume":"103","author":"M Roxo-Rosa","year":"2006","journal-title":"Proc Natl Acad Sci U S A"},{"key":"ref24","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1016\/j.jmb.2003.09.069","article-title":"Folding of Cu, Zn superoxide dismutase and familial amyotrophic lateral sclerosis.","volume":"334","author":"SD Khare","year":"2003","journal-title":"J Mol Biol"},{"key":"ref25","doi-asserted-by":"crossref","first-page":"1013","DOI":"10.1002\/bip.1981.360200512","article-title":"Non-Interacting Local-Structure Model of Folding and Unfolding Transition in Globular-Proteins .2. Application to Two-Dimensional Lattice Proteins.","volume":"20","author":"H Abe","year":"1981","journal-title":"Biopolymers"},{"key":"ref26","doi-asserted-by":"crossref","first-page":"745","DOI":"10.1016\/j.jmb.2003.12.032","article-title":"Commitment and nucleation in the protein G transition state.","volume":"336","author":"IA Hubner","year":"2004","journal-title":"J Mol Biol"}],"container-title":["PLoS Computational Biology"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/dx.plos.org\/10.1371\/journal.pcbi.1000008","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2018,10,22]],"date-time":"2018-10-22T23:59:51Z","timestamp":1540252791000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pcbi.1000008"}},"subtitle":[],"editor":[{"given":"Eugene I.","family":"Shakhnovich","sequence":"first","affiliation":[]}],"short-title":[],"issued":{"date-parts":[[2008,2,29]]},"references-count":26,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2008,2,29]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pcbi.1000008","relation":{},"ISSN":["1553-7358"],"issn-type":[{"value":"1553-7358","type":"electronic"}],"subject":[],"published":{"date-parts":[[2008,2,29]]}}}