| http://purl.uniprot.org/citations/24520399 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
| http://purl.uniprot.org/citations/24520399 | http://www.w3.org/2000/01/rdf-schema#comment | "To determine if oral dosing with the CFTR-potentiator ivacaftor (VX-770, Kalydeco) improves CFTR-dependent sweating in CF subjects carrying G551D or R117H-5T mutations, we optically measured sweat secretion from 32-143 individually identified glands in each of 8 CF subjects; 6 F508del/G551D, one G551D/R117H-5T, and one I507del/R117H-5T. Two subjects were tested only (-) ivacaftor, 3 only (+) ivacaftor and 3 (+/-) ivacaftor (1-5 tests per condition). The total number of gland measurements was 852 (-) ivacaftor and 906 (+) ivacaftor. A healthy control was tested 4 times (51 glands). For each gland we measured both CFTR-independent (M-sweat) and CFTR-dependent (C-sweat); C-sweat was stimulated with a β-adrenergic cocktail that elevated [cAMP]i while blocking muscarinic receptors. Absent ivacaftor, almost all CF glands produced M-sweat on all tests, but only 1/593 glands produced C-sweat (10 tests, 5 subjects). By contrast, 6/6 subjects (113/342 glands) produced C-sweat in the (+) ivacaftor condition, but with large inter-subject differences; 3-74% of glands responded with C/M sweat ratios 0.04%-2.57% of the average WT ratio of 0.265. Sweat volume losses cause proportionally larger underestimates of CFTR function at lower sweat rates. The losses were reduced by measuring C/M ratios in 12 glands from each subject that had the highest M-sweat rates. Remaining losses were estimated from single channel data and used to correct the C/M ratios, giving estimates of CFTR function (+) ivacaftor = 1.6%-7.7% of the WT average. These estimates are in accord with single channel data and transcript analysis, and suggest that significant clinical benefit can be produced by low levels of CFTR function."xsd:string |
| http://purl.uniprot.org/citations/24520399 | http://purl.org/dc/terms/identifier | "doi:10.1371/journal.pone.0088564"xsd:string |
| http://purl.uniprot.org/citations/24520399 | http://purl.uniprot.org/core/author | "Dunn C."xsd:string |
| http://purl.uniprot.org/citations/24520399 | http://purl.uniprot.org/core/author | "Cho H.J."xsd:string |
| http://purl.uniprot.org/citations/24520399 | http://purl.uniprot.org/core/author | "Park I.H."xsd:string |
| http://purl.uniprot.org/citations/24520399 | http://purl.uniprot.org/core/author | "Jeong J.H."xsd:string |
| http://purl.uniprot.org/citations/24520399 | http://purl.uniprot.org/core/author | "Thomas E.A."xsd:string |
| http://purl.uniprot.org/citations/24520399 | http://purl.uniprot.org/core/author | "Wine J.J."xsd:string |
| http://purl.uniprot.org/citations/24520399 | http://purl.uniprot.org/core/author | "Moss R.B."xsd:string |
| http://purl.uniprot.org/citations/24520399 | http://purl.uniprot.org/core/author | "Milla C."xsd:string |
| http://purl.uniprot.org/citations/24520399 | http://purl.uniprot.org/core/author | "Davies Z."xsd:string |
| http://purl.uniprot.org/citations/24520399 | http://purl.uniprot.org/core/author | "Char J.E."xsd:string |
| http://purl.uniprot.org/citations/24520399 | http://purl.uniprot.org/core/author | "Frisbee E."xsd:string |
| http://purl.uniprot.org/citations/24520399 | http://purl.uniprot.org/core/author | "Wolfe M.H."xsd:string |
| http://purl.uniprot.org/citations/24520399 | http://purl.uniprot.org/core/date | "2014"xsd:gYear |
| http://purl.uniprot.org/citations/24520399 | http://purl.uniprot.org/core/name | "PLoS One"xsd:string |
| http://purl.uniprot.org/citations/24520399 | http://purl.uniprot.org/core/pages | "e88564"xsd:string |
| http://purl.uniprot.org/citations/24520399 | http://purl.uniprot.org/core/title | "A little CFTR goes a long way: CFTR-dependent sweat secretion from G551D and R117H-5T cystic fibrosis subjects taking ivacaftor."xsd:string |
| http://purl.uniprot.org/citations/24520399 | http://purl.uniprot.org/core/volume | "9"xsd:string |
| http://purl.uniprot.org/citations/24520399 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/24520399 |
| http://purl.uniprot.org/citations/24520399 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/24520399 |
| http://purl.uniprot.org/uniprot/#_A0A089NFM8-mappedCitation-24520399 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/24520399 |
| http://purl.uniprot.org/uniprot/#_A0A089PPW6-mappedCitation-24520399 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/24520399 |
| http://purl.uniprot.org/uniprot/#_A0A088T1A1-mappedCitation-24520399 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/24520399 |