ライフサイエンスコーパス: cystic fibrosis

1 r degeneration (AMD), diabetes mellitus, and cystic fibrosis).

2 logy of many respiratory diseases, including cystic fibrosis.

3 mbrane regulator (CFTR) mutation that causes cystic fibrosis.

4 sweat diagnostics with reliable detection of cystic fibrosis.

5 k and lower life expectancy in patients with cystic fibrosis.

6 yperoxaluria observed in this mouse model of cystic fibrosis.

7 Cystic fibrosis.

8 ss 23 sites of the lungs from a patient with cystic fibrosis.

9 6-mediated oxalate secretion is defective in cystic fibrosis.

10 underlying cause of disease in patients with cystic fibrosis.

11 del mutation, which is the dominant cause of cystic fibrosis.

12 ted with an aggressive clinical phenotype in cystic fibrosis.

13 k and lower life expectancy in patients with cystic fibrosis.

14 function decline and increased mortality in cystic fibrosis.

15 notably prevalent among young children with cystic fibrosis.

16 for further investigation as treatments for cystic fibrosis.

17 tation that is associated with mild forms of cystic fibrosis.

18 ch is associated with the pathophysiology of cystic fibrosis.

19 een increase airway surface liquid volume in cystic fibrosis.

20 ts with hospital-acquired infections or with cystic fibrosis.

21 ic validity of attenuating IL-17 activity in cystic fibrosis.

22 s of several respiratory diseases, including cystic fibrosis.

23 of-function chloride channelopathies such as cystic fibrosis.

24 te stone formation observed in patients with cystic fibrosis.

25 ision therapies for airway disorders such as cystic fibrosis.

26 ight be promising as co-adjuvant therapy for cystic fibrosis.

27 icted to be cost-effective for patients with cystic fibrosis.

28 tricted evidence available for patients with cystic fibrosis.

29 elopathies including cardiac arrhythmias and cystic fibrosis.

30 l CRC screening strategies for patients with cystic fibrosis.

31 In cystic fibrosis, abnormal glucose tolerance is associate

32 of lumacaftor and ivacaftor in patients with cystic fibrosis aged 6-11 years homozygous for F508del-C

33 A longitudinal cohort study of patients with cystic fibrosis aged 6-21 years was conducted using the

34 Chr11p13 that is implicated as a modifier of cystic fibrosis airway disease.

35 sms fail in CFTR(-/-) swine, suggesting that cystic fibrosis airways do not respond to inhaled pathog

36 g 8 kg or more with a confirmed diagnosis of cystic fibrosis and a CFTR gating mutation on at least o

37 r developing new drugs for disorders such as cystic fibrosis and asthma.

38 ammation, and lung function in subjects with cystic fibrosis and chronic airway infections.

39 ic rhinosinusitis, and exacerbations of both cystic fibrosis and chronic obstructive pulmonary diseas

40 proved clearance in airway diseases, such as cystic fibrosis and chronic rhinosinusitis.

41 R) activity and lung function in people with cystic fibrosis and G551D-CFTR mutations but does not re

42 nts in patients aged 12 years and older with cystic fibrosis and homozygous for F508del-CFTR, but it

43 ible if they were at least 12 years old with cystic fibrosis and homozygous for the F508del-CFTR muta

44 acrophages in human lungs from patients with cystic fibrosis and induced in mouse macrophages in resp

45 ose that occur in the lungs of patients with cystic fibrosis and nonhealing ulcers.

46 teractions in A. fumigatus and patients with cystic fibrosis and the ongoing validation of novel labo

47 48 patients 12 years of age or older who had cystic fibrosis and were heterozygous for the Phe508del

48 in patients 12 years of age or older who had cystic fibrosis and were homozygous for the CFTR Phe508d

49 in patients 12 years of age or older who had cystic fibrosis and were homozygous for the CFTR Phe508d

50 lso to treat conformational diseases such as cystic fibrosis, and Alpha-1 antitrypsin deficiency.

51 te respiratory distress syndrome, pneumonia, cystic fibrosis, and bronchiectasis.

52 BACKGROUND & AIMS: Individuals with cystic fibrosis are at increased risk of colorectal canc

53 BACKGROUND & AIMS: Individuals with cystic fibrosis are at increased risk of colorectal canc

54 RATIONALE: Individuals with cystic fibrosis are at risk for prolonged drops in lung

55 Neutrophilic airway diseases, including cystic fibrosis, are characterized by excessive neutroph

56 eral nutrition-associated liver disease, and cystic fibrosis-associated liver disease.

57 onal and experimental approach to rescue the cystic-fibrosis-associated protein cystic fibrosis trans

58 ulation leads to a range of diseases such as cystic fibrosis, Bartter's syndrome and epilepsy.

59 anadian cystic fibrosis clinics and 110 U.S. cystic fibrosis care centers.

60 ombinations may have therapeutic efficacy in cystic fibrosis caused by the W1282X mutation, although

61 f its functional mechanism and correction of cystic fibrosis causing mutants.

62 The structure also reveals why many cystic-fibrosis-causing mutations would lead to defects

63 The cystic fibrosis (CF) airway surface liquid (ASL) provide

64 In cystic fibrosis (CF) altered mucus properties impair muc

65 Chronic inflammation is a hallmark of cystic fibrosis (CF) and associated with increased produ

66 They are major pathogens in patients with cystic fibrosis (CF) and can cause severe necrotizing pn

67 onization in chronic lung disease, including cystic fibrosis (CF) and chronic obstructive pulmonary d

68 mechanism in airways, and it is impaired in cystic fibrosis (CF) and other obstructive lung diseases

69 urkholderia dolosa caused an outbreak in the cystic fibrosis (CF) clinic at Boston Children's Hospita

70 erimental measurements made using normal and cystic fibrosis (CF) cultured human airway epithelium.

71 leading to hyperinflammation, a hallmark of cystic fibrosis (CF) disease.

72 RATIONALE: Individuals with cystic fibrosis (CF) experience frequent acute pulmonary

73 CFTR, the cystic fibrosis (CF) gene, encodes for the CFTR protein

74 es are reminiscent of the pathophysiology of cystic fibrosis (CF) in which loss-of-function mutations

75 progression of lung disease in children with cystic fibrosis (CF) indicates that sensitive noninvasiv

76 Cystic fibrosis (CF) is a common genetic disease caused

77 Cystic fibrosis (CF) is a major lethal genetic disease c

78 Cystic fibrosis (CF) is an autosomal recessive disorder

79 Cystic fibrosis (CF) is caused by loss-of-function mutat

80 Cystic fibrosis (CF) is caused by mutations in the gene

81 Cystic fibrosis (CF) is caused by mutations that disrupt

82 teria (NTM) from the sputum of patients with cystic fibrosis (CF) is challenging due to overgrowth by

83 Cystic fibrosis (CF) is characterized by an excessive ne

84 Cystic fibrosis (CF) is characterized by chronic infecti

85 Cystic fibrosis (CF) is characterized by early structura

86 B-OprM efflux system, naturally occurring in cystic fibrosis (CF) isolates, have been previously show

87 Cystic fibrosis (CF) liver disease (CFLD), a leading cau

88 mains an important pathogen in patients with cystic fibrosis (CF) lung disease as well as non-CF bron

89 RATIONALE: Cystic fibrosis (CF) lung disease is caused by the loss

90 ommunity of three temperate phages active in cystic fibrosis (CF) lung infections, including the tran

91 lize a panel of P. aeruginosa burn wound and cystic fibrosis (CF) lung isolates to demonstrate that P

92 respiratory virus infections predispose the cystic fibrosis (CF) lung to chronic bacterial colonizat

93 y adaptation during chronic infection of the cystic fibrosis (CF) lung, including reduced production

94 bacteria rarely reported in patients without cystic fibrosis (CF) or immunocompromising conditions.

95 istic pathogen that persists in the lungs of cystic fibrosis (CF) patients and may be responsible for

96 es chronic lung infections in the airways of cystic fibrosis (CF) patients as well as other immune-co

97 Growth failure in cystic fibrosis (CF) patients has been well-documented a

98 alence of fungi in the respiratory tracts of cystic fibrosis (CF) patients has risen.

99 e been implemented for health care visits by cystic fibrosis (CF) patients in an attempt to prevent t

100 with pulmonary exacerbations, especially in cystic fibrosis (CF) patients, and the importance of thi

101 In cystic fibrosis (CF) patients, chronic airway infection

102 the effects of NBD2 mutations identified in cystic fibrosis (CF) patients, demonstrating that mutant

103 CFTR, the chloride channel mutated in cystic fibrosis (CF) patients, is opened by ATP binding

104 pidly growing mycobacteria from the sputa of cystic fibrosis (CF) patients.

105 aeruginosa are a major cause of mortality in cystic fibrosis (CF) patients.

106 rged as a clinically significant pathogen in cystic fibrosis (CF) patients.

107 f the role of respiratory viral pathogens on cystic fibrosis (CF) pulmonary disease is needed.

108 n strategies to prevent lung damage in early cystic fibrosis (CF) requires objective outcome measures

109 Clinical manifestations of cystic fibrosis (CF) result from an increase in the visc

110 Notably, females with cystic fibrosis (CF) suffer worse outcomes, particularly

111 In the liver, the cystic fibrosis (CF) transmembrane conductance regulator

112 . aeruginosa lung infections associated with cystic fibrosis (CF) will be advanced by an improved und

113 ma membrane of secretory epithelia and cause cystic fibrosis (CF) with variable disease severity.

114 Akt signaling is suppressed in patients with cystic fibrosis (CF), a disease characterized by hyper-i

115 flammatory responses in mice and humans with cystic fibrosis (CF), a life-threatening disorder of the

116 covery of the genetic defect responsible for cystic fibrosis (CF), a monogenic disorder.

117 ole in chronic inflammatory diseases such as cystic fibrosis (CF), and targeting ER stress may be use

118 rane conductance regulator (CFTR) gene cause cystic fibrosis (CF), but are not good predictors of lun

119 sweat is an important diagnostic marker for cystic fibrosis (CF), but the implementation of point-of

120 tly in the sputum of pediatric patients with cystic fibrosis (CF), by combining the high sensitivity

121 tracellular pathogen killing is defective in cystic fibrosis (CF), despite abundant production of rea

122 ), which is defective in the genetic disease cystic fibrosis (CF), forms a gated pathway for chloride

123 important global threat to individuals with cystic fibrosis (CF), in whom M. abscessus accelerates i

124 rin in inflammatory lung diseases, including cystic fibrosis (CF), perhaps by regulation of airway su

125 ion of universal newborn screening (NBS) for cystic fibrosis (CF), the timing and magnitude of growth

126 in sepsis, pneumonia, wound infections, and cystic fibrosis (CF), which is caused by mutations of th

127 e, but their potential role in patients with cystic fibrosis (CF)-associated lung disease remains unc

128 Cystic fibrosis (CF)-related diabetes (CFRD) is thought

129 of morbidity and mortality in patients with cystic fibrosis (CF).

130 tantially increased survival of persons with cystic fibrosis (CF).

131 a (NTM) of special, international concern in Cystic Fibrosis (CF).

132 by protein kinase A, but fails to operate in cystic fibrosis (CF).

133 d gene whose loss-of-function variants cause cystic fibrosis (CF).

134 mation characterize the chronic lung disease cystic fibrosis (CF).

135 (ASL) pH has been proposed as a therapy for cystic fibrosis (CF).

136 P. aeruginosa isolates from individuals with cystic fibrosis (CF).

137 cerbations of chronic lung diseases, such as cystic fibrosis (CF).

138 cause of pulmonary disease in patients with cystic fibrosis (CF).

139 gic bronchopulmonary aspergillosis (ABPA) in cystic fibrosis (CF).

140 nosa causes lung infections in patients with cystic fibrosis (CF).

141 ve ventilation (QV) imaging in patients with cystic fibrosis (CF).

142 hogenesis and progression of lung disease in cystic fibrosis (CF).

143 fibrin gels and in sputum from patients with cystic fibrosis (CF).

144 rt in the lung, pancreas and other organs in cystic fibrosis (CF).

145 (-) channel defective in the genetic disease cystic fibrosis (CF).

146 tural lung abnormalities in individuals with cystic fibrosis (CF); however, the associations between

147 lung disease are characteristic features of cystic fibrosis (CF, OMIM #219700).

148 In addition to cystic fibrosis, CFTR dysregulation has been implicated

149 ormation of the temperature-sensitive mutant cystic fibrosis channel (F508-CFTR) at the plasma membra

150 Patients with cystic fibrosis, chronic obstructive pulmonary disease,

151 42 Canadian cystic fibrosis clinics and 110 U.S. cystic fibrosis car

152 o reducing the detrimental health effects of cystic fibrosis could be the identification of proteins

153 In cystic fibrosis, deletion of phenylalanine 508 (F508del)

154 138 mimic or siRNA against SIN3A to cultured cystic fibrosis (DeltaF508/DeltaF508) airway epithelia p

155 studies were performed in the context of the cystic fibrosis diagnosis and preliminary investigation

156 vels in artificial human sweat for potential cystic fibrosis diagnostic use.

157 t chloride is of interest as a biomarker for cystic fibrosis, electrolyte metabolism disorders, elect

158 An estimated 8%-10% of patients with cystic fibrosis experience this condition.

159 n model, we found screening of patients with cystic fibrosis for CRC to be cost effective.

160 n model, we found screening of patients with cystic fibrosis for CRC to be cost-effective.

161 a diagnosis of ABPA was the criteria of the Cystic Fibrosis Foundation Consensus Conference.

162 ian Cystic Fibrosis Registry (CCFR) and U.S. Cystic Fibrosis Foundation Patient Registry (CFFPR) betw

163 osis aged 6-21 years was conducted using the Cystic Fibrosis Foundation Patient Registry.

164 ood Institute/National Institutes of Health, Cystic Fibrosis Foundation, the University of Alabama at

165 sits in the first 12 months of life at 28 US Cystic Fibrosis Foundation-accredited Care Centers from

166 r mutant cells were grown as biofilms on the Cystic Fibrosis genotype bronchial epithelial cells.

167 muscular dystrophy, spinal muscular atrophy, cystic fibrosis, haemophilia and sickle cell disease.

168 SL secretion and whether this is abnormal in cystic fibrosis has never been tested.

169 Patients with cystic fibrosis have an increased incidence of hyperoxal

170 al therapies that target the basic defect in cystic fibrosis have recently been developed and are eff

171 ed studies and patients aged 6-11 years with cystic fibrosis homozygous for F508del-CFTR in an open-l

172 sus placebo in patients aged 6-11 years with cystic fibrosis homozygous for F508del-CFTR.

173 cacy in patients aged 12 years or older with cystic fibrosis homozygous for F508del-cystic fibrosis t

174 recting CFTR-dependent chloride transport in cystic fibrosis human airway epithelium.

175 de clinical use for the chronic treatment of cystic fibrosis in patients.

176 Median age of survival in patients with cystic fibrosis increased in both countries between 1990

177 Cystic fibrosis is a common life-limiting autosomal rece

178 Cystic fibrosis is a fatal genetic disease, most frequen

179 Cystic fibrosis is an autosomal recessive disease caused

180 to tissue remodeling and respiratory disease.Cystic fibrosis is caused by mutations in the CFTR chlor

181 Cystic fibrosis is caused by mutations in the gene encod

182 Cystic fibrosis liver disease (CFLD) in children causes

183 nstrate the in vivo contribution of IL-17 in cystic fibrosis lung disease and the therapeutic validit

184 progress independently of CFTR activity once cystic fibrosis lung disease is established.

185 ocepacia is an opportunistic pathogen of the cystic fibrosis lung that elicits a strong inflammatory

186 ile lifestyle to resilient biofilm as in the cystic fibrosis lung.

187 nome analyses of B. cenocepacia infection in cystic fibrosis lungs and serves as a valuable resource

188 s for 24 weeks in addition to their existing cystic fibrosis medications.

189 Cystic fibrosis NBS has now moved on from the developmen

190 s cathepsin C inhibitor for the treatment of cystic fibrosis, noncystic fibrosis bronchiectasis, ANCA

191 y aspergillosis occurs almost exclusively in cystic fibrosis or asthmatic patients.

192 ofilm formation, potentially contributing to cystic fibrosis pathogenesis.

193 gladioli BCC0238, a clinical isolate from a cystic fibrosis patient, led to the discovery of gladiol

194 directed approach, we were able to generate cystic fibrosis patient-specific iPSC-derived airway org

195 The bronchial cells were obtained from a cystic fibrosis patient.

196 l and chemical makeup of a human lung from a cystic fibrosis patient.

197 ed the elevated sweat electrolyte content of cystic fibrosis patients compared with that of healthy c

198 coccus aureus-specific serum IgG compared to cystic fibrosis patients despite recurrent S. aureus inf

199 idly growing mycobacteria from the sputum of cystic fibrosis patients has recently been reported.

200 ed autophagy has previously been reported in cystic fibrosis patients with the common F508del-CFTR mu

201 tant strains of P. aeruginosa (isolated from cystic fibrosis patients) indicating a potential therape

202 ed Burkholderia cenocepacia isolates from 16 cystic fibrosis patients, spanning a period of 2-20 yr a

203 leading cause of morbidity and mortality in cystic fibrosis patients.

204 ssociated with more severe meconium ileus in cystic fibrosis patients.

205 pathogen that infects immunocompromised and cystic fibrosis patients.

206 t least 15 kg, with a confirmed diagnosis of cystic fibrosis, percent predicted forced expiratory vol

207 bably the most relevant structural change in cystic fibrosis) peribronchial thickening, mucous pluggi

208 Patients with pancreatic-insufficient cystic fibrosis (PI-CF) are at increased risk for develo

209 TTs), respectively, in pancreatic-sufficient cystic fibrosis (PS-CF), PI-CF, and normal control subje

210 dam Annotated Grid Morphometric Analysis for Cystic Fibrosis quantitative outcome measure.

211 e (+0.15; 95% CI, 0.08 to 0.22; P < 0.0001), Cystic Fibrosis Questionnaire-Revised respiratory domain

212 Scores on the respiratory domain of the Cystic Fibrosis Questionnaire-Revised, a quality-of-life

213 a well-established in vivo clinical test for cystic fibrosis, reflects transepithelial cation and ani

214 Patients followed in the Canadian Cystic Fibrosis Registry (CCFR) and U.S. Cystic Fibrosis

215 Of those, two met criteria for cystic fibrosis-related diabetes, two indeterminate glyc

216 ASL secretory response to the inhalation of cystic fibrosis relevant bacteria.

217 ric muco-obstructive airway diseases such as cystic fibrosis remains unclear.

218 the median age of survival of patients with cystic fibrosis reported in the United States was 36.8 y

219 Cystic fibrosis results from mutations in the cystic fib

220 morphological abnormalities in patients with cystic fibrosis, such as bronchiectasis (which is progre

221 g-resistant pulmonary pathogen especially in cystic fibrosis sufferers.

222 retion in wild-type but not in pig models of cystic fibrosis, suggesting an impaired response to path

223 Differences in cystic fibrosis survival between Canada and the United S

224 To use a standardized approach to calculate cystic fibrosis survival estimates and to explore differ

225 3-CFTR interface might offer an approach for cystic fibrosis therapeutics.

226 caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFT

227 n autosomal recessive disorder affecting the cystic fibrosis transmembrane conductance regulator (CFT

228 modifies the local translation speed of the cystic fibrosis transmembrane conductance regulator (CFT

229 d selectivity against other proteins such as cystic fibrosis transmembrane conductance regulator (CFT

230 ase is caused by the loss of function of the cystic fibrosis transmembrane conductance regulator (CFT

231 letion of phenylalanine 508 (F508del) in the cystic fibrosis transmembrane conductance regulator (CFT

232 The cystic fibrosis transmembrane conductance regulator (CFT

233 Mutations in the cystic fibrosis transmembrane conductance regulator (CFT

234 Cystic Fibrosis Transmembrane Conductance Regulator (CFT

235 antivirals and as correctors of the F508del-cystic fibrosis transmembrane conductance regulator (CFT

236 kA), the alginate transporter (AlgE) and the cystic fibrosis transmembrane conductance regulator (CFT

237 Combination treatment with the cystic fibrosis transmembrane conductance regulator (CFT

238 Macrophages (MPhis) with mutations in cystic fibrosis transmembrane conductance regulator (CFT

239 cond nucleotide-binding domain (NBD2) of the cystic fibrosis transmembrane conductance regulator (CFT

240 ed phenylquinoxalinone CFTRact-J027 (4) as a cystic fibrosis transmembrane conductance regulator (CFT

241 ch are homologous to the gating mutations of cystic fibrosis transmembrane conductance regulator (CFT

242 Cystic fibrosis transmembrane conductance regulator (CFT

243 t is in part regulated by apically expressed cystic fibrosis transmembrane conductance regulator (CFT

244 (IL-8) secretion and decreased apical cilia, cystic fibrosis transmembrane conductance regulator (CFT

245 not dependent upon special properties of the cystic fibrosis transmembrane conductance regulator (CFT

246 e determined the amino acids inserted at the cystic fibrosis transmembrane conductance regulator (CFT

247 KEY POINTS: The cystic fibrosis transmembrane conductance regulator (CFT

248 is (CF), which is caused by mutations of the cystic fibrosis transmembrane conductance regulator (Cft

249 diseases.The F508 deletion (F508del) in the cystic fibrosis transmembrane conductance regulator (CFT

250 and specific domain interaction between the cystic fibrosis transmembrane conductance regulator (CFT

251 reased intestinal permeability and decreased cystic fibrosis transmembrane conductance regulator (Cft

252 More than 2000 mutations in the cystic fibrosis transmembrane conductance regulator (CFT

253 tive degradation of the common mutant of the cystic fibrosis transmembrane conductance regulator (CFT

254 Two functional abnormalities of cystic fibrosis transmembrane conductance regulator (CFT

255 ector, rAAV2/HBoV1, expressing a full-length cystic fibrosis transmembrane conductance regulator (CFT

256 ent protein kinase (PKG), and opening of the cystic fibrosis transmembrane conductance regulator (CFT

257 cessfully to identify the interactome of the cystic fibrosis transmembrane conductance regulator (CFT

258 Inappropriate activation of the cystic fibrosis transmembrane conductance regulator (CFT

259 n genetic disease caused by mutations of the cystic fibrosis transmembrane conductance regulator (CFT

260 irway epithelia partially restored DeltaF508-cystic fibrosis transmembrane conductance regulator (CFT

261 ng (PDE1), control of cell proliferation and cystic fibrosis transmembrane conductance regulator (CFT

262 caused by loss-of-function mutations of the cystic fibrosis transmembrane conductance regulator (CFT

263 (MRPs), and an ATP-gated anion channel, the cystic fibrosis transmembrane conductance regulator (CFT

264 ism of action of modulator compounds for the cystic fibrosis transmembrane conductance regulator (CFT

265 with cystic fibrosis homozygous for F508del-cystic fibrosis transmembrane conductance regulator (CFT

266 The cystic fibrosis transmembrane conductance regulator (CFT

267 ystic fibrosis results from mutations in the cystic fibrosis transmembrane conductance regulator (CFT

268 nstrate that mice carrying the most frequent cystic fibrosis transmembrane conductance regulator (CFT

269 Mutations in the Cystic Fibrosis Transmembrane Conductance Regulator (CFT

270 Ivacaftor is a potentiator of the cystic fibrosis transmembrane conductance regulator (CFT

271 vious work indicates that ivacaftor improves cystic fibrosis transmembrane conductance regulator (CFT

272 caused by mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator (CFT

273 ABSTRACT: Cystic fibrosis transmembrane conductance regulator (CFT

274 e expression, stability, and function of the cystic fibrosis transmembrane conductance regulator (CFT

275 The cystic fibrosis transmembrane conductance regulator (CFT

276 as I507-ATC-->ATT, in deletion of Phe508 in cystic fibrosis transmembrane conductance regulator (Del

277 d hydrostatic pressure resulted in decreased cystic fibrosis transmembrane conductance regulator acti

278 uodenal HCO3(-) secretion appears to require cystic fibrosis transmembrane conductance regulator and

279 We used mice deficient in the cystic fibrosis transmembrane conductance regulator gene

280 a secreted P. aeruginosa epoxide hydrolase, cystic fibrosis transmembrane conductance regulator inhi

281 n, an effect which was partially reversed by cystic fibrosis transmembrane conductance regulator pote

282 Cystic fibrosis transmembrane conductance regulator pote

283 To determine the feasibility of using a cystic fibrosis transmembrane conductance regulator pote

284 quently caused by the retention of the CFTR (cystic fibrosis transmembrane conductance regulator) mut

285 escue the cystic-fibrosis-associated protein cystic fibrosis transmembrane conductance regulator, whi

286 e within the airway as a result of defective cystic fibrosis transmembrane receptor (CFTR) expression

287 W1282X is the fifth most common cystic fibrosis transmembrane regulator (CFTR) mutation

288 d lower expression of chloride channel 2 and cystic fibrosis transmembrane regulator in diabetic corn

289 Gene therapy for cystic fibrosis using non-viral, plasmid-based formulati

290 say for early identification of infants with cystic fibrosis was first recognised, the performance of

291 lidation cohort included adult patients with cystic fibrosis who had CT imaging performed between Jan

292 Among patients with cystic fibrosis who had received an organ transplant, op

293 timal colonoscopy strategy for patients with cystic fibrosis who never received an organ transplant;

294 timal colonoscopy strategy for patients with cystic fibrosis who never received an organ transplant;

295 RCTs)-TRAFFIC and TRANSPORT-in patients with cystic fibrosis who were aged 12 years or older and homo

296 aftor alone was efficacious in patients with cystic fibrosis who were heterozygous for the Phe508del

297 ion therapy in patients aged 6-11 years with cystic fibrosis who were homozygous for F508del-CFTR.

298 s to be safe in children aged 2-5 years with cystic fibrosis with a gating mutation followed up for 2

299 rve for glucose and insulin in children with cystic fibrosis with control subjects.

300 t was seen predominantly in patients without cystic fibrosis with MAC and was sustained 1 year after