Electrophysiological evaluation of Cystic Fibrosis Conductance Transmembrane Regulator (CFTR) expression in human monocytes

Cystic fibrosis is caused by mutations of CFTR gene, a protein kinase A-activated anion channel, and is associated to a persistent and excessive chronic lung inflammation, suggesting functional alterations of immune cells. Leukocytes express detectable levels of CFTR but the molecule has not been fu...

Ausführliche Beschreibung

Gespeichert in:

Bibliographische Detailangaben
Veröffentlicht in:	Biochimica et biophysica acta Jg. 1840; H. 10; S. 3088 - 3095
Hauptverfasser:	Ettorre, Michele, Verzè, Genny, Caldrer, Sara, Johansson, Jan, Calcaterra, Elisa, Assael, Baroukh Maurice, Melotti, Paola, Sorio, Claudio, Buffelli, Mario
Format:	Journal Article
Sprache:	Englisch
Veröffentlicht:	Netherlands Elsevier B.V 01.10.2014
Schlagworte:	Adolescent Adult Amino Acid Sequence confocal microscopy Cystic fibrosis Cystic Fibrosis Conductance Cystic Fibrosis Transmembrane Conductance Regulator - biosynthesis Cystic Fibrosis Transmembrane Conductance Regulator - genetics electrophysiology Female fluorescence Gene Expression Regulation genes Humans inflammation Leukocytes Male Membrane Potentials Middle Aged monocytes Monocytes - cytology Monocytes - metabolism mutation Patch clamp Patch-Clamp Techniques patients protein synthesis Sequence Deletion Transmembrane Regulator CF NPD Vertex-325 Cystic Fibrosis Conductance WCR Patch clamp Cystic fibrosis S.E Leukocytes F508del ICM Transmembrane Regulator CFTRinh-172 CFTR
ISSN:	0304-4165, 0006-3002, 1872-8006
Online-Zugang:	Volltext
Tags:	Tag hinzufügen Keine Tags, Fügen Sie den ersten Tag hinzu!

Abstract	Cystic fibrosis is caused by mutations of CFTR gene, a protein kinase A-activated anion channel, and is associated to a persistent and excessive chronic lung inflammation, suggesting functional alterations of immune cells. Leukocytes express detectable levels of CFTR but the molecule has not been fully characterized in these cells. Freshly isolated monocytes from healthy individuals and CF patients were assessed by protein expression, single cell electrophysiological and membrane depolarization assays. We recorded chloride currents by patch clamp in healthy monocytes, after the administration of a CFTR stimulus. Currents were sensitive to a specific blocker of the CFTR channel, CFTRinh-172 and were absent in CF monocytes. Next, we evaluated the effects of ex vivo exposure of monocytes from cystic fibrosis patients carrying the F508del mutation to a chemical corrector, Vertex-325. We found an increase in CFTR expression by confocal microscopy and a recovery of CFTR function by both patch clamp and single cell fluorescence analysis. We confirm the expression of functional CFTR in human monocytes and demonstrate that blood monocytes can represent an adequate source of primary cells to assess new therapies and define diagnosis of CF. Tests to evaluate CFTR functional abnormalities in CF disease might greatly benefit from the availability of a convenient source of primary cells. This electrophysiological study promotes the use of monocytes as a minimally invasive tool to study and monitor CFTR function in individual patients. •Human non CF monocytes express a functional CFTR.•F508del CF monocytes recover CFTR function after ex vivo exposure to Vertex-325.•Monocytes as a possible support for diagnosis and drug development for CF
AbstractList	Cystic fibrosis is caused by mutations of CFTR gene, a protein kinase A-activated anion channel, and is associated to a persistent and excessive chronic lung inflammation, suggesting functional alterations of immune cells. Leukocytes express detectable levels of CFTR but the molecule has not been fully characterized in these cells.Freshly isolated monocytes from healthy individuals and CF patients were assessed by protein expression, single cell electrophysiological and membrane depolarization assays.We recorded chloride currents by patch clamp in healthy monocytes, after the administration of a CFTR stimulus. Currents were sensitive to a specific blocker of the CFTR channel, CFTRinh-172 and were absent in CF monocytes. Next, we evaluated the effects of ex vivo exposure of monocytes from cystic fibrosis patients carrying the F508del mutation to a chemical corrector, Vertex-325. We found an increase in CFTR expression by confocal microscopy and a recovery of CFTR function by both patch clamp and single cell fluorescence analysis.We confirm the expression of functional CFTR in human monocytes and demonstrate that blood monocytes can represent an adequate source of primary cells to assess new therapies and define diagnosis of CF.Tests to evaluate CFTR functional abnormalities in CF disease might greatly benefit from the availability of a convenient source of primary cells. This electrophysiological study promotes the use of monocytes as a minimally invasive tool to study and monitor CFTR function in individual patients. Cystic fibrosis is caused by mutations of CFTR gene, a protein kinase A-activated anion channel, and is associated to a persistent and excessive chronic lung inflammation, suggesting functional alterations of immune cells. Leukocytes express detectable levels of CFTR but the molecule has not been fully characterized in these cells.BACKGROUNDCystic fibrosis is caused by mutations of CFTR gene, a protein kinase A-activated anion channel, and is associated to a persistent and excessive chronic lung inflammation, suggesting functional alterations of immune cells. Leukocytes express detectable levels of CFTR but the molecule has not been fully characterized in these cells.Freshly isolated monocytes from healthy individuals and CF patients were assessed by protein expression, single cell electrophysiological and membrane depolarization assays.METHODSFreshly isolated monocytes from healthy individuals and CF patients were assessed by protein expression, single cell electrophysiological and membrane depolarization assays.We recorded chloride currents by patch clamp in healthy monocytes, after the administration of a CFTR stimulus. Currents were sensitive to a specific blocker of the CFTR channel, CFTRinh-172 and were absent in CF monocytes. Next, we evaluated the effects of ex vivo exposure of monocytes from cystic fibrosis patients carrying the F508del mutation to a chemical corrector, Vertex-325. We found an increase in CFTR expression by confocal microscopy and a recovery of CFTR function by both patch clamp and single cell fluorescence analysis.RESULTSWe recorded chloride currents by patch clamp in healthy monocytes, after the administration of a CFTR stimulus. Currents were sensitive to a specific blocker of the CFTR channel, CFTRinh-172 and were absent in CF monocytes. Next, we evaluated the effects of ex vivo exposure of monocytes from cystic fibrosis patients carrying the F508del mutation to a chemical corrector, Vertex-325. We found an increase in CFTR expression by confocal microscopy and a recovery of CFTR function by both patch clamp and single cell fluorescence analysis.We confirm the expression of functional CFTR in human monocytes and demonstrate that blood monocytes can represent an adequate source of primary cells to assess new therapies and define diagnosis of CF.CONCLUSIONSWe confirm the expression of functional CFTR in human monocytes and demonstrate that blood monocytes can represent an adequate source of primary cells to assess new therapies and define diagnosis of CF.Tests to evaluate CFTR functional abnormalities in CF disease might greatly benefit from the availability of a convenient source of primary cells. This electrophysiological study promotes the use of monocytes as a minimally invasive tool to study and monitor CFTR function in individual patients.GENERAL SIGNIFICANCETests to evaluate CFTR functional abnormalities in CF disease might greatly benefit from the availability of a convenient source of primary cells. This electrophysiological study promotes the use of monocytes as a minimally invasive tool to study and monitor CFTR function in individual patients. Cystic fibrosis is caused by mutations of CFTR gene, a protein kinase A-activated anion channel, and is associated to a persistent and excessive chronic lung inflammation, suggesting functional alterations of immune cells. Leukocytes express detectable levels of CFTR but the molecule has not been fully characterized in these cells. Freshly isolated monocytes from healthy individuals and CF patients were assessed by protein expression, single cell electrophysiological and membrane depolarization assays. We recorded chloride currents by patch clamp in healthy monocytes, after the administration of a CFTR stimulus. Currents were sensitive to a specific blocker of the CFTR channel, CFTRinh-172 and were absent in CF monocytes. Next, we evaluated the effects of ex vivo exposure of monocytes from cystic fibrosis patients carrying the F508del mutation to a chemical corrector, Vertex-325. We found an increase in CFTR expression by confocal microscopy and a recovery of CFTR function by both patch clamp and single cell fluorescence analysis. We confirm the expression of functional CFTR in human monocytes and demonstrate that blood monocytes can represent an adequate source of primary cells to assess new therapies and define diagnosis of CF. Tests to evaluate CFTR functional abnormalities in CF disease might greatly benefit from the availability of a convenient source of primary cells. This electrophysiological study promotes the use of monocytes as a minimally invasive tool to study and monitor CFTR function in individual patients. •Human non CF monocytes express a functional CFTR.•F508del CF monocytes recover CFTR function after ex vivo exposure to Vertex-325.•Monocytes as a possible support for diagnosis and drug development for CF Cystic fibrosis is caused by mutations of CFTR gene, a protein kinase A-activated anion channel, and is associated to a persistent and excessive chronic lung inflammation, suggesting functional alterations of immune cells. Leukocytes express detectable levels of CFTR but the molecule has not been fully characterized in these cells. Freshly isolated monocytes from healthy individuals and CF patients were assessed by protein expression, single cell electrophysiological and membrane depolarization assays. We recorded chloride currents by patch clamp in healthy monocytes, after the administration of a CFTR stimulus. Currents were sensitive to a specific blocker of the CFTR channel, CFTRinh-172 and were absent in CF monocytes. Next, we evaluated the effects of ex vivo exposure of monocytes from cystic fibrosis patients carrying the F508del mutation to a chemical corrector, Vertex-325. We found an increase in CFTR expression by confocal microscopy and a recovery of CFTR function by both patch clamp and single cell fluorescence analysis. We confirm the expression of functional CFTR in human monocytes and demonstrate that blood monocytes can represent an adequate source of primary cells to assess new therapies and define diagnosis of CF. Tests to evaluate CFTR functional abnormalities in CF disease might greatly benefit from the availability of a convenient source of primary cells. This electrophysiological study promotes the use of monocytes as a minimally invasive tool to study and monitor CFTR function in individual patients.
Author	Caldrer, Sara Sorio, Claudio Calcaterra, Elisa Johansson, Jan Assael, Baroukh Maurice Melotti, Paola Verzè, Genny Buffelli, Mario Ettorre, Michele
Author_xml	– sequence: 1 givenname: Michele surname: Ettorre fullname: Ettorre, Michele email: michele.ettorre@gmail.com organization: Department of Neurological and Movement Sciences, Section of Physiology, University of Verona, Strada Le Grazie 8, 37134 Verona, Italy – sequence: 2 givenname: Genny surname: Verzè fullname: Verzè, Genny organization: Department of Pathology and Diagnostics, University of Verona, Strada Le Grazie 8, 37134 Verona, Italy – sequence: 3 givenname: Sara surname: Caldrer fullname: Caldrer, Sara organization: Department of Pathology and Diagnostics, University of Verona, Strada Le Grazie 8, 37134 Verona, Italy – sequence: 4 givenname: Jan orcidid: 0000-0002-1671-7490 surname: Johansson fullname: Johansson, Jan organization: Department of Pathology and Diagnostics, University of Verona, Strada Le Grazie 8, 37134 Verona, Italy – sequence: 5 givenname: Elisa surname: Calcaterra fullname: Calcaterra, Elisa organization: Department of Pathology and Diagnostics, University of Verona, Strada Le Grazie 8, 37134 Verona, Italy – sequence: 6 givenname: Baroukh Maurice surname: Assael fullname: Assael, Baroukh Maurice organization: Cystic Fibrosis Center, Azienda Ospedaliera Universitaria Integrata di Verona, Piazzale Stefani 1, 37126 Verona, Italy – sequence: 7 givenname: Paola surname: Melotti fullname: Melotti, Paola organization: Cystic Fibrosis Center, Azienda Ospedaliera Universitaria Integrata di Verona, Piazzale Stefani 1, 37126 Verona, Italy – sequence: 8 givenname: Claudio surname: Sorio fullname: Sorio, Claudio organization: Department of Pathology and Diagnostics, University of Verona, Strada Le Grazie 8, 37134 Verona, Italy – sequence: 9 givenname: Mario surname: Buffelli fullname: Buffelli, Mario organization: Department of Neurological and Movement Sciences, Section of Physiology, University of Verona, Strada Le Grazie 8, 37134 Verona, Italy
BackLink	https://www.ncbi.nlm.nih.gov/pubmed/25046381$$D View this record in MEDLINE/PubMed
BookMark	eNqFkcFq3DAURUVJaSZp_6AULdOFXcmWZLuLQjGZtBAohOlayPLTRIMtTSU5dOjPR9NJNl002rzNuRdxzwU6c94BQu8pKSmh4tOuHAa1BVdWhLKSNCWh5BVa0bapipYQcYZWpCasYFTwc3QR447kxzv-Bp1XnDBRt3SF_lxPoFPw-_tDtH7yW6vVhOFBTYtK1jvsDe4PMVmN13YIPtqIe-_GRSflNOBNUC7OMA_5Ar6D7TKp5AO-6tebu48Yfu8DxHgssg7fL7NyePbO60OC-Ba9NmqK8O7pXqKf6-tN_624_XHzvf96W-i646loB82M6epOUMOYqhlVXdUwDp1gnVYwmpGOhCujqoFyJUZStUQ3tDFmaFvN6kt0derdB_9rgZjkbKOGacpf9kuUVd6lIh3ryIso5VxUbSM4z-iHJ3QZZhjlPthZhYN83jYDn0-AzrPFAEZqm_6OmoKyk6REHkXKnTyJlEeRkjQyi8xh9k_4uf-F2JdTDPKeDxaCjNpCFjXakD3L0dv_FzwCBie65g
CitedBy_id	crossref_primary_10_3390_ijms23147750 crossref_primary_10_3390_cells10123380 crossref_primary_10_1002_ppul_23712 crossref_primary_10_1371_journal_pone_0171249 crossref_primary_10_1126_scitranslmed_adk9145 crossref_primary_10_1183_13993003_03502_2020 crossref_primary_10_1164_rccm_201510_1922OC crossref_primary_10_1164_rccm_202106_1426OC crossref_primary_10_1016_j_jcf_2021_11_004 crossref_primary_10_3390_ijms25126367
Cites_doi	10.1073/pnas.1105787108 10.1164/rccm.201003-0382OC 10.1165/rcmb.2008-0170OC 10.1378/chest.124.2.482 10.1038/nbt.2328 10.1189/jlb.0412188 10.1189/jlb.0907658 10.1124/mol.110.065862 10.1126/science.2475911 10.1126/science.1234852 10.1085/jgp.201010518 10.1016/j.intimp.2008.11.011 10.1136/thx.2009.125088 10.1371/journal.pone.0022212 10.1002/cyto.a.22456 10.1016/j.jcf.2012.12.014 10.1007/BF00656997 10.1242/jeb.203.13.1947 10.1007/s002329900091 10.1111/j.1476-5381.2010.01137.x 10.1152/ajpcell.00404.2009 10.1136/thx.2005.043539 10.1152/physrev.1991.71.3.775 10.1007/s00424-009-0710-y 10.1124/mol.106.029926 10.1016/0014-5793(96)00630-8 10.1002/ppul.10043 10.1146/annurev.biochem.75.103004.142532 10.1111/j.1365-2249.2004.02635.x 10.1038/clpt.2012.183 10.1016/j.chembiol.2013.06.004 10.1016/0092-8674(90)90148-8 10.1172/JCI0216112 10.1021/bi060490t 10.1371/journal.pone.0019970 10.1016/j.jcf.2004.05.034 10.1126/science.2162561 10.1016/j.jpeds.2008.05.005 10.1183/09031936.00120910 10.1378/chest.10-2077 10.1073/pnas.0904709106 10.1038/ncb1456 10.1126/science.2570460 10.1152/ajplung.00320.2002
ContentType	Journal Article
Copyright	2014 Elsevier B.V. Copyright © 2014 Elsevier B.V. All rights reserved.
Copyright_xml	– notice: 2014 Elsevier B.V. – notice: Copyright © 2014 Elsevier B.V. All rights reserved.
DBID	AAYXX CITATION CGR CUY CVF ECM EIF NPM 7X8 7S9 L.6
DOI	10.1016/j.bbagen.2014.07.010
DatabaseName	CrossRef Medline MEDLINE MEDLINE (Ovid) MEDLINE MEDLINE PubMed MEDLINE - Academic AGRICOLA AGRICOLA - Academic
DatabaseTitle	CrossRef MEDLINE Medline Complete MEDLINE with Full Text PubMed MEDLINE (Ovid) MEDLINE - Academic AGRICOLA AGRICOLA - Academic
DatabaseTitleList	AGRICOLA MEDLINE - Academic MEDLINE
Database_xml	– sequence: 1 dbid: NPM name: PubMed url: http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed sourceTypes: Index Database – sequence: 2 dbid: 7X8 name: MEDLINE - Academic url: https://search.proquest.com/medline sourceTypes: Aggregation Database
DeliveryMethod	fulltext_linktorsrc
Discipline	Chemistry Biology
EISSN	1872-8006
EndPage	3095
ExternalDocumentID	25046381 10_1016_j_bbagen_2014_07_010 S0304416514002487
Genre	Clinical Trial Research Support, Non-U.S. Gov't Journal Article
GroupedDBID	--- --K --M .~1 0R~ 1B1 1RT 1~. 1~5 23N 3O- 4.4 457 4G. 53G 5GY 5RE 5VS 7-5 71M 8P~ 9JM AACTN AAEDT AAEDW AAIAV AAIKJ AAKOC AALRI AAOAW AAQFI AAQXK AAXUO ABEFU ABFNM ABGSF ABMAC ABUDA ABXDB ABYKQ ACDAQ ACIUM ACRLP ADBBV ADEZE ADMUD ADUVX AEBSH AEHWI AEKER AFKWA AFTJW AFXIZ AGHFR AGRDE AGUBO AGYEJ AHHHB AIEXJ AIKHN AITUG AJBFU AJOXV ALMA_UNASSIGNED_HOLDINGS AMFUW AMRAJ ASPBG AVWKF AXJTR AZFZN BKOJK BLXMC CS3 DOVZS EBS EFJIC EFLBG EJD EO8 EO9 EP2 EP3 FDB FEDTE FGOYB FIRID FNPLU FYGXN G-2 G-Q GBLVA HLW HVGLF HZ~ IHE J1W KOM LX3 M41 MO0 N9A O-L O9- OAUVE OHT OZT P-8 P-9 PC. Q38 R2- ROL RPZ SBG SCC SDF SDG SDP SES SEW SPCBC SSU SSZ T5K UQL WH7 WUQ XJT XPP ~G- 9DU AAHBH AATTM AAXKI AAYWO AAYXX ABWVN ACLOT ACRPL ACVFH ADCNI ADNMO AEIPS AEUPX AFJKZ AFPUW AGQPQ AIGII AIIUN AKBMS AKRWK AKYEP ANKPU APXCP CITATION EFKBS ~HD -~X .55 .GJ AAYJJ ABJNI AFFNX AI. CGR CUY CVF ECM EIF F5P H~9 K-O MVM NPM RIG TWZ UHS VH1 X7M Y6R YYP ZE2 ZGI ~KM 7X8 ABUFD 7S9 L.6
ID	FETCH-LOGICAL-c395t-8bc4ff93961f44a341a92745e9649caedfd1d05afa2b15a6d0280c717ffb88c43
ISICitedReferencesCount	17
ISICitedReferencesURI	http://www.webofscience.com/api/gateway?GWVersion=2&SrcApp=Summon&SrcAuth=ProQuest&DestLinkType=CitingArticles&DestApp=WOS_CPL&KeyUT=000341675900013&url=https%3A%2F%2Fcvtisr.summon.serialssolutions.com%2F%23%21%2Fsearch%3Fho%3Df%26include.ft.matches%3Dt%26l%3Dnull%26q%3D
ISSN	0304-4165 0006-3002
IngestDate	Thu Oct 02 03:42:46 EDT 2025 Sun Nov 09 14:39:30 EST 2025 Thu Apr 03 07:10:52 EDT 2025 Sat Nov 29 01:38:00 EST 2025 Tue Nov 18 22:32:14 EST 2025 Fri Feb 23 02:32:43 EST 2024
IsPeerReviewed	true
IsScholarly	true
Issue	10
Keywords	CF NPD Vertex-325 Cystic Fibrosis Conductance WCR Patch clamp Cystic fibrosis S.E Leukocytes F508del ICM Transmembrane Regulator CFTRinh-172 CFTR
Language	English
License	Copyright © 2014 Elsevier B.V. All rights reserved.
LinkModel	OpenURL
MergedId	FETCHMERGED-LOGICAL-c395t-8bc4ff93961f44a341a92745e9649caedfd1d05afa2b15a6d0280c717ffb88c43
Notes	ObjectType-Article-2 SourceType-Scholarly Journals-1 ObjectType-Feature-1 content type line 23 ObjectType-Article-1 ObjectType-Feature-2
ORCID	0000-0002-1671-7490
PMID	25046381
PQID	1556287655
PQPubID	23479
PageCount	8
ParticipantIDs	proquest_miscellaneous_2000209490 proquest_miscellaneous_1556287655 pubmed_primary_25046381 crossref_citationtrail_10_1016_j_bbagen_2014_07_010 crossref_primary_10_1016_j_bbagen_2014_07_010 elsevier_sciencedirect_doi_10_1016_j_bbagen_2014_07_010
PublicationCentury	2000
PublicationDate	2014-10-01
PublicationDateYYYYMMDD	2014-10-01
PublicationDate_xml	– month: 10 year: 2014 text: 2014-10-01 day: 01
PublicationDecade	2010
PublicationPlace	Netherlands
PublicationPlace_xml	– name: Netherlands
PublicationTitle	Biochimica et biophysica acta
PublicationTitleAlternate	Biochim Biophys Acta
PublicationYear	2014
Publisher	Elsevier B.V
Publisher_xml	– name: Elsevier B.V
References	Wong, Bear, Chin, Pasceri, Thompson, Huan, Ratjen, Ellis, Rossant (bb0070) 2012; 30 Gallin (bb0120) 1991; 71 Farrell, Rosenstein, White, Accurso, Castellani, Cutting, Durie, Legrys, Massie, Parad, Rock, Campbell (bb0210) 2008; 153 Cuthbert (bb0080) 2011; 163 Sato, Clevers (bb0065) 2013; 340 Johansson, Vezzalini, Verze, Caldrer, Bolognin, Buffelli, Bellisola, Tridello, Assael, Melotti, Sorio (bb0150) 2014; 85 Riordan (bb0005) 2008; 77 Bubien, Kirk, Rado, Frizzell (bb0170) 1990; 248 Ahrens, Standaert, Launspach, Han, Teresi, Aitken, Kelley, Hilliard, Milgram, Konstan, Weatherly, McCarty (bb0215) 2002; 33 Kim, Silver, DeCoursey (bb0130) 1996; 152 De Jonge, Ballmann, Veeze, Bronsveld, Stanke, Tummler, Sinaasappel (bb0060) 2004; 3 De Boeck, Wilschanski, Castellani, Taylor, Cuppens, Dodge, Sinaasappel (bb0205) 2006; 61 Derichs, Sanz, Von Kanel, Stolpe, Zapf, Tummler, Gallati, Ballmann (bb0055) 2010; 65 Schilling, Eder (bb0145) 2009; 459 Kim Chiaw, Wellhauser, Huan, Ramjeesingh, Bear (bb0115) 2010; 78 Wang, Loo, Bartlett, Clarke (bb0190) 2007; 71 Sermet-Gaudelus, Girodon, Sands, Stremmler, Vavrova, Deneuville, Reix, Bui, Huet, Lebourgeois, Munck, Iron, Skalicka, Bienvenu, Roussel, Lenoir, Bellon, Sarles, Macek, Roussey, Fajac, Edelman (bb0225) 2010; 182 Painter, Valentine, Lanson, Leidal, Zhang, Lombard, Thompson, Viswanathan, Nauseef, Wang, Wang (bb0045) 2006; 45 Del Porto, Cifani, Guarnieri, Di Domenico, Mariggio, Spadaro, Guglietta, Anile, Venuta, Quattrucci, Ascenzioni (bb0050) 2011; 6 Wilschanski, Miller, Shoseyov, Blau, Rivlin, Aviram, Cohen, Armoni, Yaakov, Pugatsch, Cohen-Cymberknoh, Miller, Reha, Northcutt, Hirawat, Donnelly, Elfring, Ajayi, Kerem (bb0030) 2011; 38 Farinha, King-Underwood, Sousa, Correia, Henriques, Roxo-Rosa, Da Paula, Williams, Hirst, Gomes, Amaral (bb0185) 2013; 20 Riordan, Rommens, Kerem, Alon, Rozmahel, Grzelczak, Zielenski, Lok, Plavsic, Chou (bb0105) 1989; 245 Pedemonte, Tomati, Sondo, Galietta (bb0200) 2010; 298 Cheng, Gregory, Marshall, Paul, Souza, White, O'Riordan, Smith (bb0110) 1990; 63 Kerem, Rommens, Buchanan, Markiewicz, Cox, Chakravarti, Buchwald, Tsui (bb0100) 1989; 245 Kopeikin, Sohma, Li, Hwang (bb0095) 2010; 136 Ricci, Buffelli, Riviera, Cangiano (bb0125) 1996; 390 Kim, Cheng, Agrawal (bb0135) 2004; 138 Van Goor, Hadida, Grootenhuis, Burton, Stack, Straley, Decker, Miller, McCartney, Olson, Wine, Frizzell, Ashlock, Negulescu (bb0180) 2011; 108 Shi, Wang, Liu, Jiang, Ma, Li, Zhang (bb0140) 2009; 9 Antunovic, Lukac, Vujovic (bb0020) 2013; 93 Rogan, Stoltz, Hornick (bb0010) 2011; 139 Wilschanski (bb0015) 2013; 15 Hamill, Marty, Neher, Sakmann, Sigworth (bb0085) 1981; 391 Ma, Thiagarajah, Yang, Sonawane, Folli, Galietta, Verkman (bb0090) 2002; 110 Painter, Bonvillain, Valentine, Lombard, LaPlace, Nauseef, Wang (bb0040) 2008; 83 Sorio, Angiari, Johansson, Verze, Ettorre, Buffelli, Castellani, Assael, Melotti (bb0175) 2013; 12 Bruscia, Zhang, Ferreira, Caputo, Emerson, Tuck, Krause, Egan (bb0025) 2009; 40 McCarty (bb0160) 2000; 203 Sorio, Buffelli, Angiari, Ettorre, Johansson, Vezzalini, Viviani, Ricciardi, Verze, Assael, Melotti (bb0075) 2011; 6 Brochiero, Dagenais, Prive, Berthiaume, Grygorczyk (bb0165) 2004; 287 Di, Brown, Deriy, Li, Szeto, Chen, Huang, Tong, Naren, Bindokas, Palfrey, Nelson (bb0035) 2006; 8 Bonfield, Hodges, Cotton, Drumm (bb0155) 2012; 92 Van Goor, Hadida, Grootenhuis, Burton, Cao, Neuberger, Turnbull, Singh, Joubran, Hazlewood, Zhou, McCartney, Arumugam, Decker, Yang, Young, Olson, Wine, Frizzell, Ashlock, Negulescu (bb0195) 2009; 106 Boyle, Diener-West, Milgram, Knowles, Foy, Zeitlin, Standaert (bb0220) 2003; 124 Wang (10.1016/j.bbagen.2014.07.010_bb0190) 2007; 71 Wong (10.1016/j.bbagen.2014.07.010_bb0070) 2012; 30 Ma (10.1016/j.bbagen.2014.07.010_bb0090) 2002; 110 Bonfield (10.1016/j.bbagen.2014.07.010_bb0155) 2012; 92 Kim (10.1016/j.bbagen.2014.07.010_bb0135) 2004; 138 Sorio (10.1016/j.bbagen.2014.07.010_bb0075) 2011; 6 Ricci (10.1016/j.bbagen.2014.07.010_bb0125) 1996; 390 Hamill (10.1016/j.bbagen.2014.07.010_bb0085) 1981; 391 Kopeikin (10.1016/j.bbagen.2014.07.010_bb0095) 2010; 136 Riordan (10.1016/j.bbagen.2014.07.010_bb0005) 2008; 77 Painter (10.1016/j.bbagen.2014.07.010_bb0040) 2008; 83 Rogan (10.1016/j.bbagen.2014.07.010_bb0010) 2011; 139 Kim (10.1016/j.bbagen.2014.07.010_bb0130) 1996; 152 Bruscia (10.1016/j.bbagen.2014.07.010_bb0025) 2009; 40 Boyle (10.1016/j.bbagen.2014.07.010_bb0220) 2003; 124 Del Porto (10.1016/j.bbagen.2014.07.010_bb0050) 2011; 6 Kim Chiaw (10.1016/j.bbagen.2014.07.010_bb0115) 2010; 78 Wilschanski (10.1016/j.bbagen.2014.07.010_bb0030) 2011; 38 Schilling (10.1016/j.bbagen.2014.07.010_bb0145) 2009; 459 Brochiero (10.1016/j.bbagen.2014.07.010_bb0165) 2004; 287 De Boeck (10.1016/j.bbagen.2014.07.010_bb0205) 2006; 61 Derichs (10.1016/j.bbagen.2014.07.010_bb0055) 2010; 65 Di (10.1016/j.bbagen.2014.07.010_bb0035) 2006; 8 De Jonge (10.1016/j.bbagen.2014.07.010_bb0060) 2004; 3 Wilschanski (10.1016/j.bbagen.2014.07.010_bb0015) 2013; 15 Riordan (10.1016/j.bbagen.2014.07.010_bb0105) 1989; 245 Shi (10.1016/j.bbagen.2014.07.010_bb0140) 2009; 9 Johansson (10.1016/j.bbagen.2014.07.010_bb0150) 2014; 85 Bubien (10.1016/j.bbagen.2014.07.010_bb0170) 1990; 248 Cheng (10.1016/j.bbagen.2014.07.010_bb0110) 1990; 63 Van Goor (10.1016/j.bbagen.2014.07.010_bb0180) 2011; 108 Pedemonte (10.1016/j.bbagen.2014.07.010_bb0200) 2010; 298 Gallin (10.1016/j.bbagen.2014.07.010_bb0120) 1991; 71 Farrell (10.1016/j.bbagen.2014.07.010_bb0210) 2008; 153 Sorio (10.1016/j.bbagen.2014.07.010_bb0175) 2013; 12 Sermet-Gaudelus (10.1016/j.bbagen.2014.07.010_bb0225) 2010; 182 Farinha (10.1016/j.bbagen.2014.07.010_bb0185) 2013; 20 Ahrens (10.1016/j.bbagen.2014.07.010_bb0215) 2002; 33 Sato (10.1016/j.bbagen.2014.07.010_bb0065) 2013; 340 Antunovic (10.1016/j.bbagen.2014.07.010_bb0020) 2013; 93 Painter (10.1016/j.bbagen.2014.07.010_bb0045) 2006; 45 Van Goor (10.1016/j.bbagen.2014.07.010_bb0195) 2009; 106 Cuthbert (10.1016/j.bbagen.2014.07.010_bb0080) 2011; 163 Kerem (10.1016/j.bbagen.2014.07.010_bb0100) 1989; 245 McCarty (10.1016/j.bbagen.2014.07.010_bb0160) 2000; 203
References_xml	– volume: 93 start-page: 86 year: 2013 end-page: 97 ident: bb0020 article-title: Longitudinal cystic fibrosis care publication-title: Clin. Pharmacol. Ther. – volume: 124 start-page: 482 year: 2003 end-page: 489 ident: bb0220 article-title: A multicenter study of the effect of solution temperature on nasal potential difference measurements publication-title: Chest – volume: 245 start-page: 1073 year: 1989 end-page: 1080 ident: bb0100 article-title: Identification of the cystic fibrosis gene: genetic analysis publication-title: Science – volume: 12 start-page: 821 year: 2013 end-page: 825 ident: bb0175 article-title: Impaired CFTR function in mild cystic fibrosis associated with the S977F/T5TG12complex allele in trans with F508del mutation publication-title: J. Cyst. Fibros. – volume: 6 start-page: e22212 year: 2011 ident: bb0075 article-title: Defective CFTR expression and function are detectable in blood monocytes: development of a new blood test for cystic fibrosis publication-title: PLoS One – volume: 390 start-page: 78 year: 1996 end-page: 80 ident: bb0125 article-title: An electrophysiological study of calcium entry during normal human T-lymphocyte activation publication-title: FEBS Lett. – volume: 40 start-page: 295 year: 2009 end-page: 304 ident: bb0025 article-title: Macrophages directly contribute to the exaggerated inflammatory response in cystic fibrosis transmembrane conductance regulator publication-title: Am. J. Respir. Cell Mol. Biol. – volume: 138 start-page: 453 year: 2004 end-page: 459 ident: bb0135 article-title: Cl-channels are expressed in human normal monocytes: a functional role in migration, adhesion and volume change publication-title: Clin. Exp. Immunol. – volume: 340 start-page: 1190 year: 2013 end-page: 1194 ident: bb0065 article-title: Growing self-organizing mini-guts from a single intestinal stem cell: mechanism and applications publication-title: Science – volume: 136 start-page: 659 year: 2010 end-page: 671 ident: bb0095 article-title: On the mechanism of CFTR inhibition by a thiazolidinone derivative publication-title: J. Gen. Physiol. – volume: 152 start-page: 117 year: 1996 end-page: 130 ident: bb0130 article-title: Ion channels in human THP-1 monocytes publication-title: J. Membr. Biol. – volume: 6 start-page: e19970 year: 2011 ident: bb0050 article-title: Dysfunctional CFTR alters the bactericidal activity of human macrophages against publication-title: PLoS One – volume: 38 start-page: 59 year: 2011 end-page: 69 ident: bb0030 article-title: Chronic ataluren (PTC124) treatment of nonsense mutation cystic fibrosis publication-title: Eur. Respir. J. – volume: 78 start-page: 411 year: 2010 end-page: 418 ident: bb0115 article-title: A chemical corrector modifies the channel function of F508del-CFTR publication-title: Mol. Pharmacol. – volume: 459 start-page: 71 year: 2009 end-page: 77 ident: bb0145 article-title: Lysophosphatidylcholine- and MCP-1-induced chemotaxis of monocytes requires potassium channel activity publication-title: Pflugers Arch. – volume: 203 start-page: 1947 year: 2000 end-page: 1962 ident: bb0160 article-title: Permeation through the CFTR chloride channel publication-title: J. Exp. Biol. – volume: 61 start-page: 627 year: 2006 end-page: 635 ident: bb0205 article-title: Cystic fibrosis: terminology and diagnostic algorithms publication-title: Thorax – volume: 245 start-page: 1066 year: 1989 end-page: 1073 ident: bb0105 article-title: Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA publication-title: Science – volume: 153 start-page: S4 year: 2008 end-page: S14 ident: bb0210 article-title: Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report publication-title: J. Pediatr. – volume: 77 start-page: 701 year: 2008 end-page: 726 ident: bb0005 article-title: CFTR function and prospects for therapy publication-title: Annu. Rev. Biochem. – volume: 83 start-page: 1345 year: 2008 end-page: 1353 ident: bb0040 article-title: The role of chloride anion and CFTR in killing of publication-title: J. Leukoc. Biol. – volume: 63 start-page: 827 year: 1990 end-page: 834 ident: bb0110 article-title: Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis publication-title: Cell – volume: 3 start-page: 159 year: 2004 end-page: 163 ident: bb0060 article-title: Ex vivo CF diagnosis by intestinal current measurements (ICM) in small aperture, circulating Ussing chambers publication-title: J. Cyst. Fibros. – volume: 248 start-page: 1416 year: 1990 end-page: 1419 ident: bb0170 article-title: Cell cycle dependence of chloride permeability in normal and cystic fibrosis lymphocytes publication-title: Science – volume: 8 start-page: 933 year: 2006 end-page: 944 ident: bb0035 article-title: CFTR regulates phagosome acidification in macrophages and alters bactericidal activity publication-title: Nat. Cell Biol. – volume: 9 start-page: 247 year: 2009 end-page: 252 ident: bb0140 article-title: Simvastatin inhibits acidic extracellular pH-activated, outward rectifying chloride currents in RAW264.7 monocytic-macrophage and human peripheral monocytes publication-title: Int. Immunopharmacol. – volume: 106 start-page: 18825 year: 2009 end-page: 18830 ident: bb0195 article-title: Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770 publication-title: Proc. Natl. Acad. Sci. U. S. A. – volume: 20 start-page: 943 year: 2013 end-page: 955 ident: bb0185 article-title: Revertants, low temperature, and correctors reveal the mechanism of F508del-CFTR rescue by VX-809 and suggest multiple agents for full correction publication-title: Chem. Biol. – volume: 65 start-page: 594 year: 2010 end-page: 599 ident: bb0055 article-title: Intestinal current measurement for diagnostic classification of patients with questionable cystic fibrosis: validation and reference data publication-title: Thorax – volume: 182 start-page: 929 year: 2010 end-page: 936 ident: bb0225 article-title: Clinical phenotype and genotype of children with borderline sweat test and abnormal nasal epithelial chloride transport publication-title: Am. J. Respir. Crit. Care Med. – volume: 33 start-page: 142 year: 2002 end-page: 150 ident: bb0215 article-title: Use of nasal potential difference and sweat chloride as outcome measures in multicenter clinical trials in subjects with cystic fibrosis publication-title: Pediatr. Pulmonol. – volume: 15 start-page: 127 year: 2013 end-page: 133 ident: bb0015 article-title: Novel therapeutic approaches for cystic fibrosis publication-title: Discov. Med. – volume: 298 start-page: C866 year: 2010 end-page: C874 ident: bb0200 article-title: Influence of cell background on pharmacological rescue of mutant CFTR publication-title: Am. J. Physiol. Cell Physiol. – volume: 85 start-page: 611 year: 2014 end-page: 620 ident: bb0150 article-title: Detection of CFTR protein in human leukocytes by flow cytometry publication-title: Cytometry A – volume: 163 start-page: 173 year: 2011 end-page: 183 ident: bb0080 article-title: New horizons in the treatment of cystic fibrosis publication-title: Br. J. Pharmacol. – volume: 45 start-page: 10260 year: 2006 end-page: 10269 ident: bb0045 article-title: CFTR expression in human neutrophils and the phagolysosomal chlorination defect in cystic fibrosis publication-title: Biochemistry – volume: 71 start-page: 775 year: 1991 end-page: 811 ident: bb0120 article-title: Ion channels in leukocytes publication-title: Physiol. Rev. – volume: 108 start-page: 18843 year: 2011 end-page: 18848 ident: bb0180 article-title: Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809 publication-title: Proc. Natl. Acad. Sci. U. S. A. – volume: 71 start-page: 751 year: 2007 end-page: 758 ident: bb0190 article-title: Modulating the folding of P-glycoprotein and cystic fibrosis transmembrane conductance regulator truncation mutants with pharmacological chaperones publication-title: Mol. Pharmacol. – volume: 391 start-page: 85 year: 1981 end-page: 100 ident: bb0085 article-title: Improved patch-clamp techniques for high-resolution current recording from cells and cell-free membrane patches publication-title: Pflugers Arch. – volume: 30 start-page: 876 year: 2012 end-page: 882 ident: bb0070 article-title: Directed differentiation of human pluripotent stem cells into mature airway epithelia expressing functional CFTR protein publication-title: Nat. Biotechnol. – volume: 139 start-page: 1480 year: 2011 end-page: 1490 ident: bb0010 article-title: Cystic fibrosis transmembrane conductance regulator intracellular processing, trafficking, and opportunities for mutation-specific treatment publication-title: Chest – volume: 287 start-page: L382 year: 2004 end-page: L392 ident: bb0165 article-title: Evidence of a functional CFTR Cl(−) channel in adult alveolar epithelial cells publication-title: Am. J. Physiol. Lung Cell. Mol. Physiol. – volume: 110 start-page: 1651 year: 2002 end-page: 1658 ident: bb0090 article-title: Thiazolidinone CFTR inhibitor identified by high-throughput screening blocks cholera toxin-induced intestinal fluid secretion publication-title: J. Clin. Invest. – volume: 92 start-page: 1111 year: 2012 end-page: 1122 ident: bb0155 article-title: Absence of the cystic fibrosis transmembrane regulator (CFTR) from myeloid-derived cells slows resolution of inflammation and infection publication-title: J. Leukoc. Biol. – volume: 108 start-page: 18843 year: 2011 ident: 10.1016/j.bbagen.2014.07.010_bb0180 article-title: Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809 publication-title: Proc. Natl. Acad. Sci. U. S. A. doi: 10.1073/pnas.1105787108 – volume: 182 start-page: 929 year: 2010 ident: 10.1016/j.bbagen.2014.07.010_bb0225 article-title: Clinical phenotype and genotype of children with borderline sweat test and abnormal nasal epithelial chloride transport publication-title: Am. J. Respir. Crit. Care Med. doi: 10.1164/rccm.201003-0382OC – volume: 40 start-page: 295 year: 2009 ident: 10.1016/j.bbagen.2014.07.010_bb0025 article-title: Macrophages directly contribute to the exaggerated inflammatory response in cystic fibrosis transmembrane conductance regulator−/− mice publication-title: Am. J. Respir. Cell Mol. Biol. doi: 10.1165/rcmb.2008-0170OC – volume: 124 start-page: 482 year: 2003 ident: 10.1016/j.bbagen.2014.07.010_bb0220 article-title: A multicenter study of the effect of solution temperature on nasal potential difference measurements publication-title: Chest doi: 10.1378/chest.124.2.482 – volume: 30 start-page: 876 year: 2012 ident: 10.1016/j.bbagen.2014.07.010_bb0070 article-title: Directed differentiation of human pluripotent stem cells into mature airway epithelia expressing functional CFTR protein publication-title: Nat. Biotechnol. doi: 10.1038/nbt.2328 – volume: 92 start-page: 1111 year: 2012 ident: 10.1016/j.bbagen.2014.07.010_bb0155 article-title: Absence of the cystic fibrosis transmembrane regulator (CFTR) from myeloid-derived cells slows resolution of inflammation and infection publication-title: J. Leukoc. Biol. doi: 10.1189/jlb.0412188 – volume: 83 start-page: 1345 year: 2008 ident: 10.1016/j.bbagen.2014.07.010_bb0040 article-title: The role of chloride anion and CFTR in killing of Pseudomonas aeruginosa by normal and CF neutrophils publication-title: J. Leukoc. Biol. doi: 10.1189/jlb.0907658 – volume: 78 start-page: 411 year: 2010 ident: 10.1016/j.bbagen.2014.07.010_bb0115 article-title: A chemical corrector modifies the channel function of F508del-CFTR publication-title: Mol. Pharmacol. doi: 10.1124/mol.110.065862 – volume: 245 start-page: 1066 year: 1989 ident: 10.1016/j.bbagen.2014.07.010_bb0105 article-title: Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA publication-title: Science doi: 10.1126/science.2475911 – volume: 340 start-page: 1190 year: 2013 ident: 10.1016/j.bbagen.2014.07.010_bb0065 article-title: Growing self-organizing mini-guts from a single intestinal stem cell: mechanism and applications publication-title: Science doi: 10.1126/science.1234852 – volume: 136 start-page: 659 year: 2010 ident: 10.1016/j.bbagen.2014.07.010_bb0095 article-title: On the mechanism of CFTR inhibition by a thiazolidinone derivative publication-title: J. Gen. Physiol. doi: 10.1085/jgp.201010518 – volume: 9 start-page: 247 year: 2009 ident: 10.1016/j.bbagen.2014.07.010_bb0140 article-title: Simvastatin inhibits acidic extracellular pH-activated, outward rectifying chloride currents in RAW264.7 monocytic-macrophage and human peripheral monocytes publication-title: Int. Immunopharmacol. doi: 10.1016/j.intimp.2008.11.011 – volume: 65 start-page: 594 year: 2010 ident: 10.1016/j.bbagen.2014.07.010_bb0055 article-title: Intestinal current measurement for diagnostic classification of patients with questionable cystic fibrosis: validation and reference data publication-title: Thorax doi: 10.1136/thx.2009.125088 – volume: 6 start-page: e22212 year: 2011 ident: 10.1016/j.bbagen.2014.07.010_bb0075 article-title: Defective CFTR expression and function are detectable in blood monocytes: development of a new blood test for cystic fibrosis publication-title: PLoS One doi: 10.1371/journal.pone.0022212 – volume: 85 start-page: 611 year: 2014 ident: 10.1016/j.bbagen.2014.07.010_bb0150 article-title: Detection of CFTR protein in human leukocytes by flow cytometry publication-title: Cytometry A doi: 10.1002/cyto.a.22456 – volume: 12 start-page: 821 year: 2013 ident: 10.1016/j.bbagen.2014.07.010_bb0175 article-title: Impaired CFTR function in mild cystic fibrosis associated with the S977F/T5TG12complex allele in trans with F508del mutation publication-title: J. Cyst. Fibros. doi: 10.1016/j.jcf.2012.12.014 – volume: 391 start-page: 85 year: 1981 ident: 10.1016/j.bbagen.2014.07.010_bb0085 article-title: Improved patch-clamp techniques for high-resolution current recording from cells and cell-free membrane patches publication-title: Pflugers Arch. doi: 10.1007/BF00656997 – volume: 203 start-page: 1947 year: 2000 ident: 10.1016/j.bbagen.2014.07.010_bb0160 article-title: Permeation through the CFTR chloride channel publication-title: J. Exp. Biol. doi: 10.1242/jeb.203.13.1947 – volume: 152 start-page: 117 year: 1996 ident: 10.1016/j.bbagen.2014.07.010_bb0130 article-title: Ion channels in human THP-1 monocytes publication-title: J. Membr. Biol. doi: 10.1007/s002329900091 – volume: 163 start-page: 173 year: 2011 ident: 10.1016/j.bbagen.2014.07.010_bb0080 article-title: New horizons in the treatment of cystic fibrosis publication-title: Br. J. Pharmacol. doi: 10.1111/j.1476-5381.2010.01137.x – volume: 298 start-page: C866 year: 2010 ident: 10.1016/j.bbagen.2014.07.010_bb0200 article-title: Influence of cell background on pharmacological rescue of mutant CFTR publication-title: Am. J. Physiol. Cell Physiol. doi: 10.1152/ajpcell.00404.2009 – volume: 61 start-page: 627 year: 2006 ident: 10.1016/j.bbagen.2014.07.010_bb0205 article-title: Cystic fibrosis: terminology and diagnostic algorithms publication-title: Thorax doi: 10.1136/thx.2005.043539 – volume: 71 start-page: 775 year: 1991 ident: 10.1016/j.bbagen.2014.07.010_bb0120 article-title: Ion channels in leukocytes publication-title: Physiol. Rev. doi: 10.1152/physrev.1991.71.3.775 – volume: 459 start-page: 71 year: 2009 ident: 10.1016/j.bbagen.2014.07.010_bb0145 article-title: Lysophosphatidylcholine- and MCP-1-induced chemotaxis of monocytes requires potassium channel activity publication-title: Pflugers Arch. doi: 10.1007/s00424-009-0710-y – volume: 71 start-page: 751 year: 2007 ident: 10.1016/j.bbagen.2014.07.010_bb0190 article-title: Modulating the folding of P-glycoprotein and cystic fibrosis transmembrane conductance regulator truncation mutants with pharmacological chaperones publication-title: Mol. Pharmacol. doi: 10.1124/mol.106.029926 – volume: 390 start-page: 78 year: 1996 ident: 10.1016/j.bbagen.2014.07.010_bb0125 article-title: An electrophysiological study of calcium entry during normal human T-lymphocyte activation publication-title: FEBS Lett. doi: 10.1016/0014-5793(96)00630-8 – volume: 33 start-page: 142 year: 2002 ident: 10.1016/j.bbagen.2014.07.010_bb0215 article-title: Use of nasal potential difference and sweat chloride as outcome measures in multicenter clinical trials in subjects with cystic fibrosis publication-title: Pediatr. Pulmonol. doi: 10.1002/ppul.10043 – volume: 77 start-page: 701 year: 2008 ident: 10.1016/j.bbagen.2014.07.010_bb0005 article-title: CFTR function and prospects for therapy publication-title: Annu. Rev. Biochem. doi: 10.1146/annurev.biochem.75.103004.142532 – volume: 138 start-page: 453 year: 2004 ident: 10.1016/j.bbagen.2014.07.010_bb0135 article-title: Cl-channels are expressed in human normal monocytes: a functional role in migration, adhesion and volume change publication-title: Clin. Exp. Immunol. doi: 10.1111/j.1365-2249.2004.02635.x – volume: 15 start-page: 127 year: 2013 ident: 10.1016/j.bbagen.2014.07.010_bb0015 article-title: Novel therapeutic approaches for cystic fibrosis publication-title: Discov. Med. – volume: 93 start-page: 86 year: 2013 ident: 10.1016/j.bbagen.2014.07.010_bb0020 article-title: Longitudinal cystic fibrosis care publication-title: Clin. Pharmacol. Ther. doi: 10.1038/clpt.2012.183 – volume: 20 start-page: 943 year: 2013 ident: 10.1016/j.bbagen.2014.07.010_bb0185 article-title: Revertants, low temperature, and correctors reveal the mechanism of F508del-CFTR rescue by VX-809 and suggest multiple agents for full correction publication-title: Chem. Biol. doi: 10.1016/j.chembiol.2013.06.004 – volume: 63 start-page: 827 year: 1990 ident: 10.1016/j.bbagen.2014.07.010_bb0110 article-title: Defective intracellular transport and processing of CFTR is the molecular basis of most cystic fibrosis publication-title: Cell doi: 10.1016/0092-8674(90)90148-8 – volume: 110 start-page: 1651 year: 2002 ident: 10.1016/j.bbagen.2014.07.010_bb0090 article-title: Thiazolidinone CFTR inhibitor identified by high-throughput screening blocks cholera toxin-induced intestinal fluid secretion publication-title: J. Clin. Invest. doi: 10.1172/JCI0216112 – volume: 45 start-page: 10260 year: 2006 ident: 10.1016/j.bbagen.2014.07.010_bb0045 article-title: CFTR expression in human neutrophils and the phagolysosomal chlorination defect in cystic fibrosis publication-title: Biochemistry doi: 10.1021/bi060490t – volume: 6 start-page: e19970 year: 2011 ident: 10.1016/j.bbagen.2014.07.010_bb0050 article-title: Dysfunctional CFTR alters the bactericidal activity of human macrophages against Pseudomonas aeruginosa publication-title: PLoS One doi: 10.1371/journal.pone.0019970 – volume: 3 start-page: 159 issue: Suppl. 2 year: 2004 ident: 10.1016/j.bbagen.2014.07.010_bb0060 article-title: Ex vivo CF diagnosis by intestinal current measurements (ICM) in small aperture, circulating Ussing chambers publication-title: J. Cyst. Fibros. doi: 10.1016/j.jcf.2004.05.034 – volume: 248 start-page: 1416 year: 1990 ident: 10.1016/j.bbagen.2014.07.010_bb0170 article-title: Cell cycle dependence of chloride permeability in normal and cystic fibrosis lymphocytes publication-title: Science doi: 10.1126/science.2162561 – volume: 153 start-page: S4 year: 2008 ident: 10.1016/j.bbagen.2014.07.010_bb0210 article-title: Guidelines for diagnosis of cystic fibrosis in newborns through older adults: Cystic Fibrosis Foundation consensus report publication-title: J. Pediatr. doi: 10.1016/j.jpeds.2008.05.005 – volume: 38 start-page: 59 year: 2011 ident: 10.1016/j.bbagen.2014.07.010_bb0030 article-title: Chronic ataluren (PTC124) treatment of nonsense mutation cystic fibrosis publication-title: Eur. Respir. J. doi: 10.1183/09031936.00120910 – volume: 139 start-page: 1480 year: 2011 ident: 10.1016/j.bbagen.2014.07.010_bb0010 article-title: Cystic fibrosis transmembrane conductance regulator intracellular processing, trafficking, and opportunities for mutation-specific treatment publication-title: Chest doi: 10.1378/chest.10-2077 – volume: 106 start-page: 18825 year: 2009 ident: 10.1016/j.bbagen.2014.07.010_bb0195 article-title: Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770 publication-title: Proc. Natl. Acad. Sci. U. S. A. doi: 10.1073/pnas.0904709106 – volume: 8 start-page: 933 year: 2006 ident: 10.1016/j.bbagen.2014.07.010_bb0035 article-title: CFTR regulates phagosome acidification in macrophages and alters bactericidal activity publication-title: Nat. Cell Biol. doi: 10.1038/ncb1456 – volume: 245 start-page: 1073 year: 1989 ident: 10.1016/j.bbagen.2014.07.010_bb0100 article-title: Identification of the cystic fibrosis gene: genetic analysis publication-title: Science doi: 10.1126/science.2570460 – volume: 287 start-page: L382 year: 2004 ident: 10.1016/j.bbagen.2014.07.010_bb0165 article-title: Evidence of a functional CFTR Cl(−) channel in adult alveolar epithelial cells publication-title: Am. J. Physiol. Lung Cell. Mol. Physiol. doi: 10.1152/ajplung.00320.2002
SSID	ssj0000595 ssj0025309
Score	2.2072997
Snippet	Cystic fibrosis is caused by mutations of CFTR gene, a protein kinase A-activated anion channel, and is associated to a persistent and excessive chronic lung...
SourceID	proquest pubmed crossref elsevier
SourceType	Aggregation Database Index Database Enrichment Source Publisher
StartPage	3088
SubjectTerms	Adolescent Adult Amino Acid Sequence confocal microscopy Cystic fibrosis Cystic Fibrosis Conductance Cystic Fibrosis Transmembrane Conductance Regulator - biosynthesis Cystic Fibrosis Transmembrane Conductance Regulator - genetics electrophysiology Female fluorescence Gene Expression Regulation genes Humans inflammation Leukocytes Male Membrane Potentials Middle Aged monocytes Monocytes - cytology Monocytes - metabolism mutation Patch clamp Patch-Clamp Techniques patients protein synthesis Sequence Deletion Transmembrane Regulator
Title	Electrophysiological evaluation of Cystic Fibrosis Conductance Transmembrane Regulator (CFTR) expression in human monocytes
URI	https://dx.doi.org/10.1016/j.bbagen.2014.07.010 https://www.ncbi.nlm.nih.gov/pubmed/25046381 https://www.proquest.com/docview/1556287655 https://www.proquest.com/docview/2000209490
Volume	1840
WOSCitedRecordID	wos000341675900013&url=https%3A%2F%2Fcvtisr.summon.serialssolutions.com%2F%23%21%2Fsearch%3Fho%3Df%26include.ft.matches%3Dt%26l%3Dnull%26q%3D
hasFullText	1
inHoldings	1
isFullTextHit
isPrint
journalDatabaseRights	– providerCode: PRVESC databaseName: Elsevier SD Freedom Collection Journals 2021 customDbUrl: eissn: 1872-8006 dateEnd: 99991231 omitProxy: false ssIdentifier: ssj0000595 issn: 0304-4165 databaseCode: AIEXJ dateStart: 19950118 isFulltext: true titleUrlDefault: https://www.sciencedirect.com providerName: Elsevier
link	http://cvtisr.summon.serialssolutions.com/2.0.0/link/0/eLvHCXMwtV1bb9MwFLa6DgQvCMZtXCYjIQSqUuViJ_HjVLWCaZQJdahvkZM4olOXlDadWvh5_DGO7ThpNaqNB16ixrWTOOeLfXx8zvkQeguTaBCnri9pUj2L-Kn85kLHipM0gOUzJZnQZBPBcBiOx-ys1fptYmGupkGeh6sVm_1XUUMZCFuGzv6DuOuLQgH8BqHDEcQOx1sJvq-JbZTJoh7ZmqTeyv9iLbMzdwawVC5kQpJekcu0ryp6QE1el-ISVtG55HRQVPWKhSPsDUZfpRVBrCrvWeUlqVn-oHtFsi4rj0SzSzwpku8TmZCgI8pOPNEPBWccbtY1Ka87i2UsrUGNdl-WkjPEePWLaQ2-b2L-U23th9qen-f1jkCPy6D0ubFyb3gGQX-qsLKT6lOorBwOqf3l6ugum1igPNLtkZvYnVnXg5HS8mxGNwZiWbYxqZt_r00Y2nZx0Y1jqCbz4TpEJXOtfG238nMPv0SD89PTaNQfj97NfliSukxu8Vc8Lnto3w0oC9to__hTf3zSKARUkf_UXTARnMrN8PqNd2lIu1ZAShMaPUQPqiUMPtbQe4RaIj9AdzWp6foA3esZDsHH6NffwIgbMOIiwxqM2IARb4ARb4ER12DE7yUUP-AGiHiSYwVEXAPxCTof9Ee9j1ZF92ElHqOlFcYJyTLmMd_JCOGgXnHmBoQK5hOWcJFmqZPalGfcjR3K_VR6BSSBE2RZHIYJ8Z6idl7k4jnCdsDjlDNKYmjLhcepR3yXEZ87PPVoeIg884qjpMqFLylZppFxeryItGAiKZjIDiIQzCGy6lYznQvmhvqBkV5U6bNaT40AfTe0fGOEHYHE5B4evOZiuYhA_fdd0GAo3V3HVf4FjDC4zjONlPp5ZcZCmHKdF7do_RLdbz7FV6hdzpfiNbqTXJWTxfwI7QXj8KjCOpwNzz7_AYex5cw
linkProvider	Elsevier
openUrl	ctx_ver=Z39.88-2004&ctx_enc=info%3Aofi%2Fenc%3AUTF-8&rfr_id=info%3Asid%2Fsummon.serialssolutions.com&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.atitle=Electrophysiological+evaluation+of+Cystic+Fibrosis+Conductance+Transmembrane+Regulator+%28CFTR%29+expression+in+human+monocytes&rft.jtitle=Biochimica+et+biophysica+acta.+General+subjects&rft.au=Ettorre%2C+Michele&rft.au=Verz%C3%A8%2C+Genny&rft.au=Caldrer%2C+Sara&rft.au=Johansson%2C+Jan&rft.date=2014-10-01&rft.issn=0304-4165&rft.volume=1840+p.3088-3095&rft.spage=3088&rft.epage=3095&rft_id=info:doi/10.1016%2Fj.bbagen.2014.07.010&rft.externalDBID=NO_FULL_TEXT
thumbnail_l	http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/lc.gif&issn=0304-4165&client=summon
thumbnail_m	http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/mc.gif&issn=0304-4165&client=summon
thumbnail_s	http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/sc.gif&issn=0304-4165&client=summon