Defective CFTR Expression and Function Are Detectable in Blood Monocytes: Development of a New Blood Test for Cystic Fibrosis

Evaluation of cystic fibrosis transmembrane conductance regulator (CFTR) functional activity to assess new therapies and define diagnosis of cystic fibrosis (CF) is cumbersome. It is known that leukocytes express detectable levels of CFTR but the molecule has not been characterized in these cells. I...

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Vydané v:PloS one Ročník 6; číslo 7; s. e22212
Hlavní autori: Sorio, Claudio, Buffelli, Mario, Angiari, Chiara, Ettorre, Michele, Johansson, Jan, Vezzalini, Marzia, Viviani, Laura, Ricciardi, Mario, Verzè, Genny, Assael, Baroukh Maurice, Melotti, Paola
Médium: Journal Article
Jazyk:English
Vydavateľské údaje: United States Public Library of Science 21.07.2011
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ISSN:1932-6203, 1932-6203
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Abstract Evaluation of cystic fibrosis transmembrane conductance regulator (CFTR) functional activity to assess new therapies and define diagnosis of cystic fibrosis (CF) is cumbersome. It is known that leukocytes express detectable levels of CFTR but the molecule has not been characterized in these cells. In this study we aim at setting up and validating a blood test to evaluate CFTR expression and function in leukocytes. Western blot, PCR, immunofluorescence and cell membrane depolarization analysis by single-cell fluorescence imaging, using the potential-sensitive DiSBAC(2)(3) probe were utilized. Expression of PKA phosphorylated, cell membrane-localized CFTR was detected in non-CF monocytes, being undetectable or present in truncated form in monocytes derived from CF patients presenting with nonsense mutations. CFTR agonist administration induced membrane depolarization in monocytes isolated from non-CF donors (31 subjects) and, to a lesser extent, obligate CFTR heterozygous carriers (HTZ: 15 subjects), but it failed in monocytes from CF patients (44 subjects). We propose an index, which values in CF patients are significantly (p<0.001) lower than in the other two groups. Nasal Potential Difference, measured in selected subjects had concordant results with monocytes assay (Kappa statistic 0.93, 95%CI: 0.80-1.00). CFTR is detectable and is functional in human monocytes. We also showed that CFTR-associated activity can be evaluated in 5 ml of peripheral blood and devise an index potentially applicable for diagnostic purposes and both basic and translational research: from drug development to evaluation of functional outcomes in clinical trials.
AbstractList Background Evaluation of cystic fibrosis transmembrane conductance regulator (CFTR) functional activity to assess new therapies and define diagnosis of cystic fibrosis (CF) is cumbersome. It is known that leukocytes express detectable levels of CFTR but the molecule has not been characterized in these cells. In this study we aim at setting up and validating a blood test to evaluate CFTR expression and function in leukocytes. Description Western blot, PCR, immunofluorescence and cell membrane depolarization analysis by single-cell fluorescence imaging, using the potential-sensitive DiSBAC.sub.2 (3) probe were utilized. Expression of PKA phosphorylated, cell membrane-localized CFTR was detected in non-CF monocytes, being undetectable or present in truncated form in monocytes derived from CF patients presenting with nonsense mutations. CFTR agonist administration induced membrane depolarization in monocytes isolated from non-CF donors (31 subjects) and, to a lesser extent, obligate CFTR heterozygous carriers (HTZ: 15 subjects), but it failed in monocytes from CF patients (44 subjects). We propose an index, which values in CF patients are significantly (p<0.001) lower than in the other two groups. Nasal Potential Difference, measured in selected subjects had concordant results with monocytes assay (Kappa statistic 0.93, 95%CI: 0.80-1.00). Results and Significance CFTR is detectable and is functional in human monocytes. We also showed that CFTR-associated activity can be evaluated in 5 ml of peripheral blood and devise an index potentially applicable for diagnostic purposes and both basic and translational research: from drug development to evaluation of functional outcomes in clinical trials.
Description CFTR is detectable and is functional in human monocytes. We also showed that CFTR-associated activity can be evaluated in 5 ml of peripheral blood and devise an index potentially applicable for diagnostic purposes and both basic and translational research: from drug development to evaluation of functional outcomes in clinical trials.
Background Evaluation of cystic fibrosis transmembrane conductance regulator (CFTR) functional activity to assess new therapies and define diagnosis of cystic fibrosis (CF) is cumbersome. It is known that leukocytes express detectable levels of CFTR but the molecule has not been characterized in these cells. In this study we aim at setting up and validating a blood test to evaluate CFTR expression and function in leukocytes. Description Western blot, PCR, immunofluorescence and cell membrane depolarization analysis by single-cell fluorescence imaging, using the potential-sensitive DiSBAC2(3) probe were utilized. Expression of PKA phosphorylated, cell membrane-localized CFTR was detected in non-CF monocytes, being undetectable or present in truncated form in monocytes derived from CF patients presenting with nonsense mutations. CFTR agonist administration induced membrane depolarization in monocytes isolated from non-CF donors (31 subjects) and, to a lesser extent, obligate CFTR heterozygous carriers (HTZ: 15 subjects), but it failed in monocytes from CF patients (44 subjects). We propose an index, which values in CF patients are significantly (p<0.001) lower than in the other two groups. Nasal Potential Difference, measured in selected subjects had concordant results with monocytes assay (Kappa statistic 0.93, 95%CI: 0.80–1.00). Results and Significance CFTR is detectable and is functional in human monocytes. We also showed that CFTR-associated activity can be evaluated in 5 ml of peripheral blood and devise an index potentially applicable for diagnostic purposes and both basic and translational research: from drug development to evaluation of functional outcomes in clinical trials.
Evaluation of cystic fibrosis transmembrane conductance regulator (CFTR) functional activity to assess new therapies and define diagnosis of cystic fibrosis (CF) is cumbersome. It is known that leukocytes express detectable levels of CFTR but the molecule has not been characterized in these cells. In this study we aim at setting up and validating a blood test to evaluate CFTR expression and function in leukocytes. Western blot, PCR, immunofluorescence and cell membrane depolarization analysis by single-cell fluorescence imaging, using the potential-sensitive DiSBAC(2)(3) probe were utilized. Expression of PKA phosphorylated, cell membrane-localized CFTR was detected in non-CF monocytes, being undetectable or present in truncated form in monocytes derived from CF patients presenting with nonsense mutations. CFTR agonist administration induced membrane depolarization in monocytes isolated from non-CF donors (31 subjects) and, to a lesser extent, obligate CFTR heterozygous carriers (HTZ: 15 subjects), but it failed in monocytes from CF patients (44 subjects). We propose an index, which values in CF patients are significantly (p<0.001) lower than in the other two groups. Nasal Potential Difference, measured in selected subjects had concordant results with monocytes assay (Kappa statistic 0.93, 95%CI: 0.80-1.00). CFTR is detectable and is functional in human monocytes. We also showed that CFTR-associated activity can be evaluated in 5 ml of peripheral blood and devise an index potentially applicable for diagnostic purposes and both basic and translational research: from drug development to evaluation of functional outcomes in clinical trials.
Evaluation of cystic fibrosis transmembrane conductance regulator (CFTR) functional activity to assess new therapies and define diagnosis of cystic fibrosis (CF) is cumbersome. It is known that leukocytes express detectable levels of CFTR but the molecule has not been characterized in these cells. In this study we aim at setting up and validating a blood test to evaluate CFTR expression and function in leukocytes.BACKGROUNDEvaluation of cystic fibrosis transmembrane conductance regulator (CFTR) functional activity to assess new therapies and define diagnosis of cystic fibrosis (CF) is cumbersome. It is known that leukocytes express detectable levels of CFTR but the molecule has not been characterized in these cells. In this study we aim at setting up and validating a blood test to evaluate CFTR expression and function in leukocytes.Western blot, PCR, immunofluorescence and cell membrane depolarization analysis by single-cell fluorescence imaging, using the potential-sensitive DiSBAC(2)(3) probe were utilized. Expression of PKA phosphorylated, cell membrane-localized CFTR was detected in non-CF monocytes, being undetectable or present in truncated form in monocytes derived from CF patients presenting with nonsense mutations. CFTR agonist administration induced membrane depolarization in monocytes isolated from non-CF donors (31 subjects) and, to a lesser extent, obligate CFTR heterozygous carriers (HTZ: 15 subjects), but it failed in monocytes from CF patients (44 subjects). We propose an index, which values in CF patients are significantly (p<0.001) lower than in the other two groups. Nasal Potential Difference, measured in selected subjects had concordant results with monocytes assay (Kappa statistic 0.93, 95%CI: 0.80-1.00).DESCRIPTIONWestern blot, PCR, immunofluorescence and cell membrane depolarization analysis by single-cell fluorescence imaging, using the potential-sensitive DiSBAC(2)(3) probe were utilized. Expression of PKA phosphorylated, cell membrane-localized CFTR was detected in non-CF monocytes, being undetectable or present in truncated form in monocytes derived from CF patients presenting with nonsense mutations. CFTR agonist administration induced membrane depolarization in monocytes isolated from non-CF donors (31 subjects) and, to a lesser extent, obligate CFTR heterozygous carriers (HTZ: 15 subjects), but it failed in monocytes from CF patients (44 subjects). We propose an index, which values in CF patients are significantly (p<0.001) lower than in the other two groups. Nasal Potential Difference, measured in selected subjects had concordant results with monocytes assay (Kappa statistic 0.93, 95%CI: 0.80-1.00).CFTR is detectable and is functional in human monocytes. We also showed that CFTR-associated activity can be evaluated in 5 ml of peripheral blood and devise an index potentially applicable for diagnostic purposes and both basic and translational research: from drug development to evaluation of functional outcomes in clinical trials.RESULTS AND SIGNIFICANCECFTR is detectable and is functional in human monocytes. We also showed that CFTR-associated activity can be evaluated in 5 ml of peripheral blood and devise an index potentially applicable for diagnostic purposes and both basic and translational research: from drug development to evaluation of functional outcomes in clinical trials.
Background Evaluation of cystic fibrosis transmembrane conductance regulator (CFTR) functional activity to assess new therapies and define diagnosis of cystic fibrosis (CF) is cumbersome. It is known that leukocytes express detectable levels of CFTR but the molecule has not been characterized in these cells. In this study we aim at setting up and validating a blood test to evaluate CFTR expression and function in leukocytes. Description Western blot, PCR, immunofluorescence and cell membrane depolarization analysis by single-cell fluorescence imaging, using the potential-sensitive DiSBAC2(3) probe were utilized. Expression of PKA phosphorylated, cell membrane-localized CFTR was detected in non-CF monocytes, being undetectable or present in truncated form in monocytes derived from CF patients presenting with nonsense mutations. CFTR agonist administration induced membrane depolarization in monocytes isolated from non-CF donors (31 subjects) and, to a lesser extent, obligate CFTR heterozygous carriers (HTZ: 15 subjects), but it failed in monocytes from CF patients (44 subjects). We propose an index, which values in CF patients are significantly (p<0.001) lower than in the other two groups. Nasal Potential Difference, measured in selected subjects had concordant results with monocytes assay (Kappa statistic 0.93, 95%CI: 0.80–1.00). Results and Significance CFTR is detectable and is functional in human monocytes. We also showed that CFTR-associated activity can be evaluated in 5 ml of peripheral blood and devise an index potentially applicable for diagnostic purposes and both basic and translational research: from drug development to evaluation of functional outcomes in clinical trials.
BackgroundEvaluation of cystic fibrosis transmembrane conductance regulator (CFTR) functional activity to assess new therapies and define diagnosis of cystic fibrosis (CF) is cumbersome. It is known that leukocytes express detectable levels of CFTR but the molecule has not been characterized in these cells. In this study we aim at setting up and validating a blood test to evaluate CFTR expression and function in leukocytes.DescriptionWestern blot, PCR, immunofluorescence and cell membrane depolarization analysis by single-cell fluorescence imaging, using the potential-sensitive DiSBAC(2)(3) probe were utilized. Expression of PKA phosphorylated, cell membrane-localized CFTR was detected in non-CF monocytes, being undetectable or present in truncated form in monocytes derived from CF patients presenting with nonsense mutations. CFTR agonist administration induced membrane depolarization in monocytes isolated from non-CF donors (31 subjects) and, to a lesser extent, obligate CFTR heterozygous carriers (HTZ: 15 subjects), but it failed in monocytes from CF patients (44 subjects). We propose an index, which values in CF patients are significantly (p<0.001) lower than in the other two groups. Nasal Potential Difference, measured in selected subjects had concordant results with monocytes assay (Kappa statistic 0.93, 95%CI: 0.80-1.00).Results and significanceCFTR is detectable and is functional in human monocytes. We also showed that CFTR-associated activity can be evaluated in 5 ml of peripheral blood and devise an index potentially applicable for diagnostic purposes and both basic and translational research: from drug development to evaluation of functional outcomes in clinical trials.
Audience Academic
Author Sorio, Claudio
Johansson, Jan
Vezzalini, Marzia
Assael, Baroukh Maurice
Viviani, Laura
Angiari, Chiara
Ricciardi, Mario
Melotti, Paola
Verzè, Genny
Buffelli, Mario
Ettorre, Michele
AuthorAffiliation Comprehensive Pneumology Center, Germany
4 Sezione di Statistica Medica e Biometria “G.A. Maccacaro”, Dipartimento di Medicina del Lavoro “Clinica del Lavoro L. Devoto”, Università degli Studi di Milano, Milano, Italy
1 Sezione di Patologia Generale, Dipartimento di Patologia e Diagnostica, University of Verona, Verona, Italy
2 Physiology Section, Dipartimento di Scienze Neurologiche, Neuropsicologiche Morfologiche e Motorie, University of Verona, Verona, Italy
3 Centro Fibrosi Cistica, Azienda Ospedaliera Universitaria Integrata di Verona, Verona, Italy
AuthorAffiliation_xml – name: 3 Centro Fibrosi Cistica, Azienda Ospedaliera Universitaria Integrata di Verona, Verona, Italy
– name: Comprehensive Pneumology Center, Germany
– name: 2 Physiology Section, Dipartimento di Scienze Neurologiche, Neuropsicologiche Morfologiche e Motorie, University of Verona, Verona, Italy
– name: 4 Sezione di Statistica Medica e Biometria “G.A. Maccacaro”, Dipartimento di Medicina del Lavoro “Clinica del Lavoro L. Devoto”, Università degli Studi di Milano, Milano, Italy
– name: 1 Sezione di Patologia Generale, Dipartimento di Patologia e Diagnostica, University of Verona, Verona, Italy
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  surname: Sorio
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  surname: Melotti
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BackLink https://www.ncbi.nlm.nih.gov/pubmed/21811577$$D View this record in MEDLINE/PubMed
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Cites_doi 10.1016/j.jcf.2004.05.034
10.1073/pnas.87.10.4012
10.1016/0092-8674(93)90353-R
10.1021/bi060490t
10.1016/S0140-6736(08)61168-X
10.1042/BJ20080029
10.1164/ajrccm/151.3_Pt_1.899
10.1165/ajrcmb.10.1.7507342
10.1378/chest.06-2975
10.1016/S0008-6363(95)00245-6
10.4049/jimmunol.0902851
10.1378/chest.07-0288
10.1016/j.cell.2010.11.029
10.1136/thx.2005.043539
10.1002/ppul.10043
10.1164/rccm.200509-1377OC
10.1111/j.1476-5381.1991.tb09842.x
10.1136/thx.2009.125088
10.1258/000456307779596011
10.1034/j.1399-3003.2000.16d30.x
10.1093/nar/19.19.5417
10.1038/ncb1456
10.1089/hum.1995.6.10-1275
10.1146/annurev.biochem.75.103004.142532
10.1006/geno.1998.5517
10.1038/ejhg.2008.136
10.1038/sj.gt.3301741
10.1164/rccm.201003-0382OC
10.1016/0895-4356(90)90159-M
10.1016/S0006-3495(96)79508-5
10.1378/chest.124.2.482
10.1016/S0021-9258(19)50722-0
10.1007/978-1-4613-8122-8
10.1016/j.jcf.2004.05.032
10.1165/ajrcmb/4.4.313
10.1189/jlb.0309120
10.1016/j.jpeds.2008.05.005
10.1159/000091457
10.1183/09031936.01.00092501
ContentType Journal Article
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2011 Sorio et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License: https://creativecommons.org/licenses/by/4.0/ (the “License”), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.
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Conceived and designed the experiments: CS PM. Performed the experiments: CA JJ ME GV MV MR. Analyzed the data: CA JJ LV ME MB. Contributed reagents/materials/analysis tools: BMA PM LV MB. Wrote the paper: CS PM.
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References JR Riordan (ref1) 2008; 77
Y Zhang (ref39) 2010; 184
E Bruscia (ref10) 2002; 9
M Renier (ref17) 1995; 6
A Green (ref7) 2007; 44
K Yoshimura (ref2) 1991; 19
RC Ahrens (ref19) 2002; 33
A Di (ref9) 2006; 8
C Sorio (ref15) 1995; 55
M Wilschanski (ref31) 2006; 174
SJ Mason (ref43) 1991; 103
T Iwamura (ref13) 1987; 78
G Blom (ref22) 1958
J Xie (ref26) 1996; 71
E Kerem (ref41) 2008; 372
HR De Jonge (ref4) 2004; 3
PL Zeitlin (ref12) 1991; 4
SH Donaldson (ref29) 2007; 132
AGM Bot (ref25) 2002
E Dequeker (ref38) 2009; 17
RG Painter (ref24) 2006; 45
JD Warth (ref16) 1996; 31
DV Cicchetti (ref28) 1990; 43
A Stumpf (ref8) 2006; 17
EM Bruscia (ref40) 2008
K De Boeck (ref35) 2006; 61
D Schuler (ref20) 2004; 3
MJ Welsh (ref42) 1993; 73
C Sorio (ref33) 2009; 86
N Derichs (ref32) 2010; 65
M Wilschanski (ref27) 2001; 17
JH Chen (ref30) 2010; 143
O Chiba-Falek (ref34) 1998; 53
AL Cozens (ref11) 1994; 10
E Caci (ref18) 2008; 413
Y Yaakov (ref5) 2007; 132
MP Boyle (ref21) 2003; 124
I Sermet-Gaudelus (ref37) 2010; 182
G Doring (ref44) 2000; 16
TV McDonald (ref3) 1992; 267
RGJ Miller (ref23) 1981
B Stewart (ref6) 1995; 151
PM Farrell (ref36) 2008; 153
RA Schoumacher (ref14) 1990; 87
2189948 - J Clin Epidemiol. 1990;43(6):551-8
7585520 - Cancer Res. 1995 Nov 1;55(21):4855-64
16543719 - Cell Physiol Biochem. 2006;17(1-2):29-36
16922501 - Biochemistry. 2006 Aug 29;45(34):10260-9
16384879 - Thorax. 2006 Jul;61(7):627-35
11106223 - Eur Respir J. 2000 Oct;16(4):749-67
19720614 - J Leukoc Biol. 2009 Sep;86(3):465-8
7533604 - Am J Respir Crit Care Med. 1995 Mar;151(3 Pt 1):899-903
11491166 - Eur Respir J. 2001 Jun;17(6):1208-15
20627915 - Thorax. 2010 Jul;65(7):594-9
7686820 - Cell. 1993 Jul 2;73(7):1251-4
17998363 - Chest. 2007 Nov;132(5):1631-6
18722008 - Lancet. 2008 Aug 30;372(9640):719-27
8968585 - Biophys J. 1996 Dec;71(6):3148-56
21145458 - Cell. 2010 Dec 10;143(6):911-23
20351190 - J Immunol. 2010 May 1;184(9):5104-11
8689654 - Cardiovasc Res. 1996 Apr;31(4):615-24
1371114 - J Biol Chem. 1992 Feb 15;267(5):3242-8
17890478 - Chest. 2007 Oct;132(4):1219-26
17270089 - Ann Clin Biochem. 2007 Jan;44(Pt 1):25-34
16840743 - Am J Respir Crit Care Med. 2006 Oct 1;174(7):787-94
11802252 - Pediatr Pulmonol. 2002 Feb;33(2):142-50
12032687 - Gene Ther. 2002 Jun;9(11):683-5
3102439 - Jpn J Cancer Res. 1987 Jan;78(1):54-62
1692630 - Proc Natl Acad Sci U S A. 1990 May;87(10):4012-6
12907532 - Chest. 2003 Aug;124(2):482-9
1718521 - Br J Pharmacol. 1991 Jul;103(3):1649-56
20538955 - Am J Respir Crit Care Med. 2010 Oct 1;182(7):929-36
9799593 - Genomics. 1998 Nov 1;53(3):276-83
18366345 - Biochem J. 2008 Jul 1;413(1):135-42
7507342 - Am J Respir Cell Mol Biol. 1994 Jan;10(1):38-47
16921366 - Nat Cell Biol. 2006 Sep;8(9):933-44
15463949 - J Cyst Fibros. 2004 Aug;3 Suppl 2:151-5
8590731 - Hum Gene Ther. 1995 Oct;6(10):1275-83
1717947 - Nucleic Acids Res. 1991 Oct 11;19(19):5417-23
18685558 - Eur J Hum Genet. 2009 Jan;17(1):51-65
15463951 - J Cyst Fibros. 2004 Aug;3 Suppl 2:159-63
1849726 - Am J Respir Cell Mol Biol. 1991 Apr;4(4):313-9
18639722 - J Pediatr. 2008 Aug;153(2):S4-S14
18304008 - Annu Rev Biochem. 2008;77:701-26
18776130 - Am J Respir Cell Mol Biol. 2009 Mar;40(3):295-304
References_xml – volume: 3
  start-page: 159
  year: 2004
  ident: ref4
  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: 87
  start-page: 4012
  year: 1990
  ident: ref14
  article-title: A cystic fibrosis pancreatic adenocarcinoma cell line.
  publication-title: Proc Natl Acad Sci U S A
  doi: 10.1073/pnas.87.10.4012
– volume: 78
  start-page: 54
  year: 1987
  ident: ref13
  article-title: Establishment and characterization of a human pancreatic cancer cell line (SUIT-2) producing carcinoembryonic antigen and carbohydrate antigen 19-9.
  publication-title: Jpn J Cancer Res
– volume: 73
  start-page: 1251
  year: 1993
  ident: ref42
  article-title: Molecular mechanisms of CFTR chloride channel dysfunction in cystic fibrosis.
  publication-title: Cell
  doi: 10.1016/0092-8674(93)90353-R
– volume: 45
  start-page: 10260
  year: 2006
  ident: ref24
  article-title: CFTR Expression in human neutrophils and the phagolysosomal chlorination defect in cystic fibrosis.
  publication-title: Biochemistry
  doi: 10.1021/bi060490t
– volume: 372
  start-page: 719
  year: 2008
  ident: ref41
  article-title: Effectiveness of PTC124 treatment of cystic fibrosis caused by nonsense mutations: a prospective phase II trial.
  publication-title: Lancet
  doi: 10.1016/S0140-6736(08)61168-X
– year: 2002
  ident: ref25
  article-title: Immunodetection of CFTR protein in human and mouse tissues by Western blotting.
– volume: 413
  start-page: 135
  year: 2008
  ident: ref18
  article-title: Evidence for direct CFTR inhibition by CFTR(inh)-172 based on Arg347 mutagenesis.
  publication-title: Biochem J
  doi: 10.1042/BJ20080029
– volume: 151
  start-page: 899
  year: 1995
  ident: ref6
  article-title: Normal sweat chloride values do not exclude the diagnosis of cystic fibrosis.
  publication-title: Am J Respir Crit Care Med
  doi: 10.1164/ajrccm/151.3_Pt_1.899
– volume: 10
  start-page: 38
  year: 1994
  ident: ref11
  article-title: CFTR expression and chloride secretion in polarized immortal human bronchial epithelial cells.
  publication-title: Am J Respir Cell Mol Biol
  doi: 10.1165/ajrcmb.10.1.7507342
– volume: 132
  start-page: 1219
  year: 2007
  ident: ref5
  article-title: Reproducibility of nasal potential difference measurements in cystic fibrosis.
  publication-title: Chest
  doi: 10.1378/chest.06-2975
– volume: 31
  start-page: 615
  year: 1996
  ident: ref16
  article-title: CFTR chloride channels in human and simian heart.
  publication-title: Cardiovasc Res
  doi: 10.1016/S0008-6363(95)00245-6
– volume: 184
  start-page: 5104
  year: 2010
  ident: ref39
  article-title: Alterations in ceramide concentration and pH determine the release of reactive oxygen species by Cftr-deficient macrophages on infection.
  publication-title: J Immunol
  doi: 10.4049/jimmunol.0902851
– volume: 132
  start-page: 1631
  year: 2007
  ident: ref29
  article-title: Sodium channels and cystic fibrosis.
  publication-title: Chest
  doi: 10.1378/chest.07-0288
– volume: 143
  start-page: 911
  year: 2010
  ident: ref30
  article-title: Loss of anion transport without increased sodium absorption characterizes newborn porcine cystic fibrosis airway epithelia.
  publication-title: Cell
  doi: 10.1016/j.cell.2010.11.029
– volume: 61
  start-page: 627
  year: 2006
  ident: ref35
  article-title: Cystic fibrosis: terminology and diagnostic algorithms.
  publication-title: Thorax
  doi: 10.1136/thx.2005.043539
– volume: 55
  start-page: 4855
  year: 1995
  ident: ref15
  article-title: Characterization of the receptor protein tyrosine phosphatase gene product PTP gamma: binding and activation by triphosphorylated nucleosides.
  publication-title: Cancer Res
– year: 2008
  ident: ref40
  article-title: Macrophages Directly Contribute to the Exaggerated Inflammatory Response in CFTR−/− mice.
  publication-title: Am J Respir Cell Mol Biol
– volume: 33
  start-page: 142
  year: 2002
  ident: ref19
  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: 174
  start-page: 787
  year: 2006
  ident: ref31
  article-title: Mutations in the cystic fibrosis transmembrane regulator gene and in vivo transepithelial potentials.
  publication-title: Am J Respir Crit Care Med
  doi: 10.1164/rccm.200509-1377OC
– volume: 103
  start-page: 1649
  year: 1991
  ident: ref43
  article-title: Regulation of transepithelial ion transport and intracellular calcium by extracellular ATP in human normal and cystic fibrosis airway epithelium.
  publication-title: Br J Pharmacol
  doi: 10.1111/j.1476-5381.1991.tb09842.x
– volume: 65
  start-page: 594
  year: 2010
  ident: ref32
  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: 44
  start-page: 25
  year: 2007
  ident: ref7
  article-title: Guidelines for the performance of the sweat test for the diagnosis of cystic fibrosis.
  publication-title: Ann Clin Biochem
  doi: 10.1258/000456307779596011
– volume: 16
  start-page: 749
  year: 2000
  ident: ref44
  article-title: Antibiotic therapy against Pseudomonas aeruginosa in cystic fibrosis: a European consensus.
  publication-title: Eur Respir J
  doi: 10.1034/j.1399-3003.2000.16d30.x
– volume: 19
  start-page: 5417
  year: 1991
  ident: ref2
  article-title: Expression of the cystic fibrosis transmembrane conductance regulator gene in cells of non-epithelial origin.
  publication-title: Nucleic Acids Res
  doi: 10.1093/nar/19.19.5417
– volume: 8
  start-page: 933
  year: 2006
  ident: ref9
  article-title: CFTR regulates phagosome acidification in macrophages and alters bactericidal activity.
  publication-title: Nat Cell Biol
  doi: 10.1038/ncb1456
– volume: 6
  start-page: 1275
  year: 1995
  ident: ref17
  article-title: Use of a membrane potential-sensitive probe to assess biological expression of the cystic fibrosis transmembrane conductance regulator.
  publication-title: Hum Gene Ther
  doi: 10.1089/hum.1995.6.10-1275
– volume: 77
  start-page: 701
  year: 2008
  ident: ref1
  article-title: CFTR function and prospects for therapy.
  publication-title: Annu Rev Biochem
  doi: 10.1146/annurev.biochem.75.103004.142532
– volume: 53
  start-page: 276
  year: 1998
  ident: ref34
  article-title: The molecular basis of disease variability among cystic fibrosis patients carrying the 3849+10 kb C→T mutation.
  publication-title: Genomics
  doi: 10.1006/geno.1998.5517
– volume: 17
  start-page: 51
  year: 2009
  ident: ref38
  article-title: Best practice guidelines for molecular genetic diagnosis of cystic fibrosis and CFTR-related disorders–updated European recommendations.
  publication-title: Eur J Hum Genet
  doi: 10.1038/ejhg.2008.136
– volume: 9
  start-page: 683
  year: 2002
  ident: ref10
  article-title: Isolation of CF cell lines corrected at DeltaF508-CFTR locus by SFHR-mediated targeting.
  publication-title: Gene Ther
  doi: 10.1038/sj.gt.3301741
– volume: 182
  start-page: 929
  year: 2010
  ident: ref37
  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: 43
  start-page: 551
  year: 1990
  ident: ref28
  article-title: High agreement but low kappa: II. Resolving the paradoxes.
  publication-title: J Clin Epidemiol
  doi: 10.1016/0895-4356(90)90159-M
– year: 1958
  ident: ref22
  article-title: Statistical Estimates and Transformed Beta Variables
– volume: 71
  start-page: 3148
  year: 1996
  ident: ref26
  article-title: Human epithelial cystic fibrosis transmembrane conductance regulator without exon 5 maintains partial chloride channel function in intracellular membranes.
  publication-title: Biophys J
  doi: 10.1016/S0006-3495(96)79508-5
– volume: 124
  start-page: 482
  year: 2003
  ident: ref21
  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: 267
  start-page: 3242
  year: 1992
  ident: ref3
  article-title: Human lymphocytes transcribe the cystic fibrosis transmembrane conductance regulator gene and exhibit CF-defective cAMP-regulated chloride current.
  publication-title: J Biol Chem
  doi: 10.1016/S0021-9258(19)50722-0
– year: 1981
  ident: ref23
  article-title: Simultaneous Statistical Inference
  doi: 10.1007/978-1-4613-8122-8
– volume: 3
  start-page: 151
  year: 2004
  ident: ref20
  article-title: Basic protocol for transepithelial nasal potential difference measurements.
  publication-title: J Cyst Fibros
  doi: 10.1016/j.jcf.2004.05.032
– volume: 4
  start-page: 313
  year: 1991
  ident: ref12
  article-title: A cystic fibrosis bronchial epithelial cell line: immortalization by adeno-12-SV40 infection.
  publication-title: Am J Respir Cell Mol Biol
  doi: 10.1165/ajrcmb/4.4.313
– volume: 86
  start-page: 465
  year: 2009
  ident: ref33
  article-title: Editorial: The role of macrophages and their scavenger receptors in cystic fibrosis.
  publication-title: J Leukoc Biol
  doi: 10.1189/jlb.0309120
– volume: 153
  start-page: S4
  year: 2008
  ident: ref36
  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: 17
  start-page: 29
  year: 2006
  ident: ref8
  article-title: Physiological concept for a blood based CFTR test.
  publication-title: Cell Physiol Biochem
  doi: 10.1159/000091457
– volume: 17
  start-page: 1208
  year: 2001
  ident: ref27
  article-title: Nasal potential difference measurements in patients with atypical cystic fibrosis.
  publication-title: Eur Respir J
  doi: 10.1183/09031936.01.00092501
– reference: 7507342 - Am J Respir Cell Mol Biol. 1994 Jan;10(1):38-47
– reference: 18685558 - Eur J Hum Genet. 2009 Jan;17(1):51-65
– reference: 8590731 - Hum Gene Ther. 1995 Oct;6(10):1275-83
– reference: 16922501 - Biochemistry. 2006 Aug 29;45(34):10260-9
– reference: 2189948 - J Clin Epidemiol. 1990;43(6):551-8
– reference: 1718521 - Br J Pharmacol. 1991 Jul;103(3):1649-56
– reference: 7533604 - Am J Respir Crit Care Med. 1995 Mar;151(3 Pt 1):899-903
– reference: 16543719 - Cell Physiol Biochem. 2006;17(1-2):29-36
– reference: 16840743 - Am J Respir Crit Care Med. 2006 Oct 1;174(7):787-94
– reference: 11491166 - Eur Respir J. 2001 Jun;17(6):1208-15
– reference: 8689654 - Cardiovasc Res. 1996 Apr;31(4):615-24
– reference: 18722008 - Lancet. 2008 Aug 30;372(9640):719-27
– reference: 15463951 - J Cyst Fibros. 2004 Aug;3 Suppl 2:159-63
– reference: 1849726 - Am J Respir Cell Mol Biol. 1991 Apr;4(4):313-9
– reference: 17270089 - Ann Clin Biochem. 2007 Jan;44(Pt 1):25-34
– reference: 18304008 - Annu Rev Biochem. 2008;77:701-26
– reference: 21145458 - Cell. 2010 Dec 10;143(6):911-23
– reference: 19720614 - J Leukoc Biol. 2009 Sep;86(3):465-8
– reference: 15463949 - J Cyst Fibros. 2004 Aug;3 Suppl 2:151-5
– reference: 16384879 - Thorax. 2006 Jul;61(7):627-35
– reference: 11106223 - Eur Respir J. 2000 Oct;16(4):749-67
– reference: 1371114 - J Biol Chem. 1992 Feb 15;267(5):3242-8
– reference: 12032687 - Gene Ther. 2002 Jun;9(11):683-5
– reference: 8968585 - Biophys J. 1996 Dec;71(6):3148-56
– reference: 7686820 - Cell. 1993 Jul 2;73(7):1251-4
– reference: 20351190 - J Immunol. 2010 May 1;184(9):5104-11
– reference: 1692630 - Proc Natl Acad Sci U S A. 1990 May;87(10):4012-6
– reference: 20627915 - Thorax. 2010 Jul;65(7):594-9
– reference: 17998363 - Chest. 2007 Nov;132(5):1631-6
– reference: 9799593 - Genomics. 1998 Nov 1;53(3):276-83
– reference: 16921366 - Nat Cell Biol. 2006 Sep;8(9):933-44
– reference: 17890478 - Chest. 2007 Oct;132(4):1219-26
– reference: 18776130 - Am J Respir Cell Mol Biol. 2009 Mar;40(3):295-304
– reference: 11802252 - Pediatr Pulmonol. 2002 Feb;33(2):142-50
– reference: 18639722 - J Pediatr. 2008 Aug;153(2):S4-S14
– reference: 7585520 - Cancer Res. 1995 Nov 1;55(21):4855-64
– reference: 12907532 - Chest. 2003 Aug;124(2):482-9
– reference: 18366345 - Biochem J. 2008 Jul 1;413(1):135-42
– reference: 1717947 - Nucleic Acids Res. 1991 Oct 11;19(19):5417-23
– reference: 20538955 - Am J Respir Crit Care Med. 2010 Oct 1;182(7):929-36
– reference: 3102439 - Jpn J Cancer Res. 1987 Jan;78(1):54-62
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Snippet Evaluation of cystic fibrosis transmembrane conductance regulator (CFTR) functional activity to assess new therapies and define diagnosis of cystic fibrosis...
Background Evaluation of cystic fibrosis transmembrane conductance regulator (CFTR) functional activity to assess new therapies and define diagnosis of cystic...
Description CFTR is detectable and is functional in human monocytes. We also showed that CFTR-associated activity can be evaluated in 5 ml of peripheral blood...
BackgroundEvaluation of cystic fibrosis transmembrane conductance regulator (CFTR) functional activity to assess new therapies and define diagnosis of cystic...
Background Evaluation of cystic fibrosis transmembrane conductance regulator (CFTR) functional activity to assess new therapies and define diagnosis of cystic...
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StartPage e22212
SubjectTerms Adolescent
Adult
Aged
Algorithms
Amiloride - pharmacology
Biology
Blood
Blood tests
Cell Line
Child
Child, Preschool
Chloride
Clinical trials
Conductance
Cystic fibrosis
Cystic Fibrosis - blood
Cystic Fibrosis - diagnosis
Cystic Fibrosis - physiopathology
Cystic Fibrosis Transmembrane Conductance Regulator - blood
Cystic Fibrosis Transmembrane Conductance Regulator - metabolism
Depolarization
Diagnosis
Diagnostic systems
Drug development
Epithelial Cells - drug effects
Epithelial Cells - metabolism
Evaluation
Female
Fibrosis
Fluorescence
Genetic counseling
Genotype & phenotype
Hematologic Tests - methods
Heterozygote
Humans
Immunofluorescence
Indexing
Infant
Kinases
Leukocytes
Male
Medical research
Medicine
Membrane potential
Membrane Potentials - drug effects
Middle Aged
Monocytes
Monocytes - drug effects
Monocytes - metabolism
Mutation
Older people
Patients
Peripheral blood
Phenotype
Physiology
Protein kinase A
Pseudomonas aeruginosa
Resistance
Single-Cell Analysis
White blood cells
Young Adult
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Title Defective CFTR Expression and Function Are Detectable in Blood Monocytes: Development of a New Blood Test for Cystic Fibrosis
URI https://www.ncbi.nlm.nih.gov/pubmed/21811577
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