title: Nasal potential difference measurements to assess CFTR ion channel activity.
abstract: The Nasal potential difference measurement is used to measure the voltage across the nasal epithelium, which results from transepithelial ion transport and reflects in part CFTR function. The electrophysiologic abnormality in cystic fibrosis was first described 30 years ago and correlates with features of the CF phenotype. NPD measurement is an important in vivo research and diagnostic tool, and is used to assess the efficacy of new treatments such as gene therapy and ion transport modulators. This chapter will elaborate on the electrophysiological principles behind the test, the equipment required, the methods, and the analysis of the data.
authors: Department of Medicine, University of Alabama, Birmingham, AL 35294-0006, USA. smrowe@uab.edu
authors: Steven M
authors: SM
authors: Rowe
authors: John Paul
authors: JP
authors: Clancy
authors: Michael
authors: M
authors: Wilschanski
references: Proc Am Thorac Soc. 2007 Aug 1;4(4):387-98
references: 17652506
references: Chest. 2007 Oct;132(4):1219-26
references: 17890478
references: Eur Respir J. 1994 Nov;7(11):2050-6
references: 7875281
references: Am Rev Respir Dis. 1981 Oct;124(4):484-90
references: 7294508
references: Hum Gene Ther. 2004 Dec;15(12):1255-69
references: 15684701
references: N Engl J Med. 2005 May 12;352(19):1992-2001
references: 15888700
references: Eur Respir J. 1994 Mar;7(3):442-5
references: 8013599
references: BMC Med. 2007 Mar 29;5:5
references: 17394637
references: Hum Gene Ther. 1995 Apr;6(4):445-55
references: 7542031
references: Am J Respir Crit Care Med. 2006 Oct 1;174(7):787-94
references: 16840743
references: Thorax. 2006 Jul;61(7):627-35
references: 16384879
references: Pediatr Pulmonol. 2002 Feb;33(2):90-8
references: 11802244
references: Eur Respir J. 2001 Jun;17(6):1208-15
references: 11491166
references: N Engl J Med. 1981 Dec 17;305(25):1489-95
references: 7300874
references: Pediatr Pulmonol. 2002 Feb;33(2):142-50
references: 11802252
references: Am J Respir Cell Mol Biol. 2007 Jul;37(1):57-66
references: 17347447
references: Pharmacol Ther. 2005 Nov;108(2):208-23
references: 15936089
references: N Engl J Med. 1995 Sep 28;333(13):823-31
references: 7544439
references: Mol Ther. 2000 Jan;1(1):105-14
references: 10933918
references: Am Rev Respir Dis. 1982 Jul;126(1):108-12
references: 7091895
references: Am J Physiol. 1967 Aug;213(2):425-31
references: 4962440
references: Thorax. 2004 Nov;59(11):971-6
references: 15516474
references: N Engl J Med. 2003 Oct 9;349(15):1433-41
references: 14534336
references: Science. 1991 Jul 12;253(5016):202-5
references: 1712984
references: Clin Chem Lab Med. 2003 Jan;41(1):61-7
references: 12636051
references: Am J Respir Crit Care Med. 2005 May 1;171(9):1015-9
references: 15640364
references: Am J Respir Crit Care Med. 1996 Jul;154(1):217-23
references: 8680683
references: Chest. 2004 Jan;125(1):143-9
references: 14718433
references: J Pediatr. 2008 Aug;153(2):S4-S14
references: 18639722