ライフサイエンスコーパス: CF

1 CF is characterized by repeated lung infections leading

2 CF MDMs demonstrate a nearly 60% reduction in superoxide

3 CF patients under corrector-potentiator therapy, which e

4 CF photographs of 30 eyes (20 patients) with diabetic ma

5 CF was inversely associated with clinical attachment lev

6 CF-ALF influences the P. aeruginosa cell wall by reducin

7 CF-related growth deficits can be improved via inhibitio

8 al dependency graph from approximately 3,000 CF-MS experiments on human cell lines.

9 F% = 56-110%) and mavacoxib (F% = 111-113%), CF and standard solutions, respectively.

10 e collected from respiratory cultures of 115 CF patients during a 22-month study period.

11 ducted a multicenter, randomized trial at 14 CF centers with subjects at least 14 years old.

12 le KL levels were measured in plasma from 48 CF patients and in primary CF bronchial epithelial cells

13 A total of 487 CF respiratory samples were collected from 211 unique pa

14 is indicated by individual findings), and a CF-specific intensive bowel preparation.

15 Importantly, we show that activated CF MPhis have reduced protein levels and altered localiz

16 s from 33 individuals: 11 with T2D and acute CF, 11 T2D patients with equivalent neuropathy and 11 no

17 re significantly increased in MPs from acute CF patients.

18 ed the prevalence of these fungi in an adult CF population.

19 plitude compared to patches containing adult CFs, while pure mESC-CM patches did not form functional

20 nic CFs is superior to co-culture with adult CFs for in vitro generation of functional myocardium.

21 large collection (n = 333) of P. aeruginosa CF isolates.

22 Although CF MDMs generally have increased total NADPH component p

23 s suggest a therapeutic avenue to ameliorate CF-associated bacterial infections.

24 ence, we conclude that CS30 is common among "CF negative" LT + STp isolates and is associated with ET

25 In this article, we analyze CF and non-CF human monocyte-derived macrophages (MDMs)

26 lthy (n = 49), lung cancer (LC) (n = 31) and CF (n = 31) subjects.

27 persion field and compensation field (DF and CF), and mass-to-charge (m/z).

28 lar interactions through CF...S, CF...H, and CF...pi noncovalent interactions and enhance electron mo

29 bronchial epithelial cells from healthy and CF donors was assessed by surface biotinylation and subs

30 se levels between human ALF from healthy and CF patients differ.

31 predicted ASL height under basal normal and CF conditions and the collapse of surface hydration due

32 , and calcium release in response to PMA and CF pathogens.

33 onses to P. aeruginosa We used wild-type and CF mice, which we hormone manipulated, inoculated with P

34 3 (Meg3) was found to be mostly expressed by CFs and to undergo transcriptional downregulation during

35 ine mole fraction of lysine plus cadaverine (CF) indicated biofilm lysine decarboxylase activity.

36 nd in primary CF bronchial epithelial cells (CF-HBEC).

37 tion of toxic byproducts such as chloroform (CF).

38 e common groundwater contaminant chloroform (CF), this study investigated for the first time both car

39 instability of F508del-CFTR, the most common CF mutation.

40 d of the gating defects caused by two common CF mutations.

41 designated anthocyanins (AF) and copigments (CF), was carried out using adsorptive membrane chromatog

42 etween Mediator and the RSC, Arp2/Arp3, CPF, CF 1A and Lsm complexes in chromatin.

43 n/coconut fibre/zinc oxide nanoparticles (CS/CF/nZnO) hybrid support to yield a polyamine sensing str

44 m based on chlorophyll fluorescence DCA (DCA-CF), and static controlled atmosphere.

45 orage based on chlorophyll fluorescence (DCA-CF) and respiratory quotient (DCA-RQ) on the quality and

46 tate), as compared to fruit stored under DCA-CF, but fruit stored under DCA-RQ 1.5 and RQ 2.0 also sh

47 es ranked with student t-test discriminating CF antigens from healthy controls and LC at a False Disc

48 Of note, the current assemblies of the dog, CF 3.1 and the sheep, OA 3.1, genomes contain 264 and 59

49 Th1 effector cells selectively drive CF both in vitro and in vivo, whereas adoptive transfer

50 ltidrug approach is required for efficacious CF therapy.

51 hat 3D co-culture of mESC-CMs with embryonic CFs is superior to co-culture with adult CFs for in vitr

52 ac6 knockout mice were crossed with F508del (CF) mice to generate F508del/Hdac6 (CF/HDA) mice.

53 (IUs) as compared to contracted facilities (CFs).

54 ntigenically different colonization factors (CFs) have been identified and characterized in ETEC at l

55 e structures, known as colonization factors (CFs).

56 c juice and sputa from sixteen adult PEG fed CF patients and five replaced PEG tubes were studied.

57 Fetal CF-conditioned media distinctly enhanced CM spreading an

58 Tissue patches containing fetal CFs exhibited higher velocity of action potential propag

59 ted with media conditioned by adult or fetal CFs.

60 ntal changes may induce cardiac fibroblasts (CF) pathological responses is far from being elucidated,

61 on assays, we show that cardiac fibroblasts (CFs) adopt an osteoblast cell-like fate and contribute d

62 RATIONALE: Cardiac fibroblasts (CFs) drive extracellular matrix remodeling after pressur

63 opmental stage of mouse cardiac fibroblasts (CFs) influences the formation and function of engineered

64 roblasts (CFBs) to promote cardiac fibrosis (CF) in nonischemic heart failure (HF).

65 In cystic fibrosis (CF) altered mucus properties impair mucociliary transpor

66 flammation is a hallmark of cystic fibrosis (CF) and associated with increased production of transfor

67 pathogens in patients with cystic fibrosis (CF) and can cause severe necrotizing pneumonia, which is

68 ments made using normal and cystic fibrosis (CF) cultured human airway epithelium.

69 RATIONALE: Individuals with cystic fibrosis (CF) experience frequent acute pulmonary exacerbations, w

70 ng disease in children with cystic fibrosis (CF) indicates that sensitive noninvasive outcome measure

71 Cystic fibrosis (CF) is an autosomal recessive disorder affecting the cys

72 Cystic fibrosis (CF) is caused by mutations in the gene encoding the cyst

73 Cystic fibrosis (CF) is caused by mutations that disrupt the plasma membr

74 tem, naturally occurring in cystic fibrosis (CF) isolates, have been previously shown to reverse this

75 Cystic fibrosis (CF) liver disease (CFLD), a leading cause of death in CF

76 RATIONALE: Cystic fibrosis (CF) lung disease is caused by the loss of function of th

77 eported in patients without cystic fibrosis (CF) or immunocompromising conditions.

78 n the respiratory tracts of cystic fibrosis (CF) patients has risen.

79 In cystic fibrosis (CF) patients, chronic airway infection by Pseudomonas le

80 BD2 mutations identified in cystic fibrosis (CF) patients, demonstrating that mutants such as N1303K

81 major cause of mortality in cystic fibrosis (CF) patients.

82 lly significant pathogen in cystic fibrosis (CF) patients.

83 piratory viral pathogens on cystic fibrosis (CF) pulmonary disease is needed.

84 flammatory diseases such as cystic fibrosis (CF), and targeting ER stress may be useful for alleviati

85 regulator (CFTR) gene cause cystic fibrosis (CF), but are not good predictors of lung phenotype.

86 rtant diagnostic marker for cystic fibrosis (CF), but the implementation of point-of-care systems for

87 gen killing is defective in cystic fibrosis (CF), despite abundant production of reactive oxygen spec

88 newborn screening (NBS) for cystic fibrosis (CF), the timing and magnitude of growth deficiency or it

89 onia, wound infections, and cystic fibrosis (CF), which is caused by mutations of the cystic fibrosis

90 Cystic fibrosis (CF)-related diabetes (CFRD) is thought to result from be

91 gression of lung disease in cystic fibrosis (CF).

92 ary aspergillosis (ABPA) in cystic fibrosis (CF).

93 infections in patients with cystic fibrosis (CF).

94 ancreas and other organs in cystic fibrosis (CF).

95 V) imaging in patients with cystic fibrosis (CF).

96 n sputum from patients with cystic fibrosis (CF).

97 tive in the genetic disease cystic fibrosis (CF).

98 mortality in patients with cystic fibrosis (CF).

99 ed survival of persons with cystic fibrosis (CF).

100 l, international concern in Cystic Fibrosis (CF).

101 ze the chronic lung disease cystic fibrosis (CF).

102 ompared with non-CF, MUC5B more often filled CF submucosal gland ducts.

103 abase representing the corrugated flamelets (CF), thin reaction zones (TRZ) and broken reaction zones

104 edictive of mortality after continuous flow (CF) left ventricular assist device (LVAD) implantation.

105 ve two cycles of cisplatin and fluorouracil (CF; two 3-weekly cycles of cisplatin [80 mg/m(2) intrave

106 (PPW), retinal folds (RF), choroidal folds (CF), and creases using transaxial and en face views.

107 however, their contribution to Charcot foot (CF) disease is not known.

108 single-molecule-based therapeutic agent for CF.

109 iotropic beneficial effects is available for CF.

110 8del-CFTR subpopulation might be crucial for CF therapy development.

111 Since initiation of universal NBS for CF, significant improvement has occurred in nutritional

112 way for point-of-care diagnostic systems for CF.

113 ing it as a potential therapeutic target for CF.

114 of atrial fibrillation after contact force (CF)-guided pulmonary vein isolation.

115 administration of a commercial formulation (CF).

116 and at 12 months and grouped as caries free (CF), caries active with enamel lesions (CAE), and caries

117 HF patients, as well as the protection from CF and HF in TCR-alpha(-/-) mice.

118 re able to detect PQS in sputum samples from CF patients infected with P. aeruginosa but not in sampl

119 he area of HEs in the digital colour fundus (CF) photographs.

120 F508del (CF) mice to generate F508del/Hdac6 (CF/HDA) mice.

121 of seven genes, csmA-G, related to the human CF operon CS18 and the porcine CF operon 987P (F6).

122 However, it is unknown whether human CF macrophages have an independent defect in the oxidati

123 EC isolates lacked any previously identified CF.

124 Finally, we identified CF specific clones that correlate highly with sweat chlo

125 after chronic pressure overload to identify CF-enriched lncRNAs and investigate their function and c

126 lication, power, impedance drop [Delta-Imp], CF, force-time integral [FTI], and ablation index [AI])

127 In CF-guided pulmonary vein isolation, PVR is explained by

128 In CF-HBEC, TGF-beta increased KL secretion and upregulated

129 indings support a role for CXCL1 and IL-8 in CF lung disease severity and identify STAT3 as a modulat

130 c and sensitive for the diagnosis of ABPA in CF.

131 ccus sanguinis, which was highly abundant in CF-PF.

132 demonstrated reduced tubulin acetylation in CF cell models and tissue that is correctable by inhibit

133 channel openings, the dysfunction of CFTR in CF and the action of drugs that repair CFTR gating defec

134 disease (CFLD), a leading cause of death in CF, is mostly described in pediatric populations.

135 sens the overall oxidative burst deficits in CF MDMs compared with non-CF MDMs.

136 poron species and Exophiala dermatitidis, in CF sputa using standard bacterial and selective fungal c

137 , an effect that could exacerbate disease in CF and COPD patients.

138 irus infections have a prolonged duration in CF.

139 ignificant deterioration of lung function in CF over time.

140 The prevalence of fungi in CF may be better estimated by using selective fungal cul

141 s may explain the sex-based mortality gap in CF and other inflammatory lung illnesses, and the ER blo

142 neral population, and 25-30 times greater in CF patients after an organ transplantation.

143 development of postprandial hyperglycemia in CF.

144 cytes and monocytes origin were increased in CF patients.

145 deria species can cause serious infection in CF and themselves affect key oxidase components in murin

146 ells and prevents P. aeruginosa infection in CF mice.

147 aeruginosa from chronic airway infection in CF patients.

148 Pulmonary S. aureus infections in CF often occur very early and prior to colonization with

149 iating damaging neutrophilic inflammation in CF airways.

150 y of the pathogenetic mechanisms involved in CF, which include impaired chloride permeability and per

151 egrin distribution and sphingosine levels in CF epithelial cells and prevents P. aeruginosa infection

152 intermediate and non-classical monocytes in CF patients.

153 , our study suggests that circulating MPs in CF disease have a high content of inflammatory cytokines

154 I3K activity, similar to what is observed in CF.

155 Pseudomonas aeruginosa culture positivity in CF patients with unclear mechanisms.

156 on of ENaC may have therapeutic potential in CF airways by reducing sodium hyperabsorption, restoring

157 hypothesis that such changes are present in CF pancreata before the development of lipofibrosis.

158 However, evidence that ASL pH is reduced in CF is limited and conflicting.

159 modulate TGF beta-induced IL-8 secretion in CF bronchial epithelia.

160 This observation is also true in CF animal models, including mouse, pig, rat, and ferret.

161 Variability in CF decreased while a change from an exponential to a pow

162 GapmeR-mediated silencing of Meg3 in CFs resulted in the downregulation of Mmp-2 transcriptio

163 in) is able to additionally induce increased CF release of inflammatory and pro-fibrotic cytokines an

164 may allow the pathogen to chronically infect CF lungs.

165 ast 30% of clinical ETEC isolates lack known CFs.

166 arches with the structural subunits of known CFs identified 6 new putative CF variants.

167 Our large CF population-based cohort demonstrated a significant as

168 ents had >/=10 mg/dL differences between LDL-CF and LDL-CD compared with 25% and 20% of patients, res

169 ents had differences >/=10 mg/dL between LDL-CF and LDL-CD, whereas only 2% and 3% of patients, respe

170 proach of the classic Friedewald method (LDL-CF).

171 ing within an estimated LDL-C (LDL-CN or LDL-CF) category by clinical cut points.

172 en LDL-CD and estimated LDL-C (LDL-CN or LDL-CF) was stratified by LDL-C and triglyceride categories.

173 <70 mg/dL accuracy (82%) was superior to LDL-CF (37%; P<0.001).

174 ng LDL-CN accuracy (92%) was superior to LDL-CF accuracy (71%; P<0.001).

175 Following LT, CF-adapted Pseudomonas strains, potentially originating

176 us attention on the role of EHF in modifying CF lung disease severity.

177 In mESC-CM monolayers, CF-conditioned media did not alter CM spreading or MEK-E

178 t characterizing lncRNA expression in murine CFs after chronic pressure overload to identify CF-enric

179 In this article, we analyze CF and non-CF human monocyte-derived macrophages (MDMs) for ROS pro

180 to our understanding of Bcc infection in non-CF patients and highlight the need for interventions to

181 affect key oxidase components in murine non-CF cells.

182 dstream infections (BSIs) in a cohort of non-CF patients from the US Veterans Health Administration (

183 de the donor microbiota and adapt to the non-CF allograft.

184 tion after PMA stimulation compared with non-CF MDMs.

185 (phox) and p40 (phox) in comparison with non-CF MDMs.

186 burst deficits in CF MDMs compared with non-CF MDMs.

187 Compared with non-CF, MUC5B more often filled CF submucosal gland ducts.

188 ar macrophages into the lungs of noninfected CF mice is sufficient to induce pneumonia.

189 We identified a novel CF, CS30, encoded by a set of seven genes, csmA-G, relat

190 CF formation with only approximately 10% of CF recovery while sarcosine (SAR) showed insignificant e

191 Correlation analysis of CF-MS profiles shows promise in detecting protein comple

192 agonists to effectively rehydrate the ASL of CF airways.

193 phylococcus aureus isolates from a cohort of CF patients as assessed by strain relatedness.

194 A cohort of CF patients without CFLD during childhood were followed

195 neoadjuvant ECX compared with two cycles of CF did not increase survival, and cannot be considered s

196 d) that completed the molecular diagnosis of CF in 6 of 14 individuals.

197 ghly symmetric non-Gaussian distributions of CF support zero-sum dynamics.

198 aC mouse model as well as the sheep model of CF.

199 ole for NBD2 instability in the pathology of CF.

200 icroF cm(-2) ) from combined polarization of CF interface dipoles and electrical-double-layer formati

201 ., healthy children) from the progression of CF lung disease.

202 table MICs on a non-negligible proportion of CF isolates, explained by a wide diversity of mutations

203 R) all resulted in remarkable suppression of CF formation with only approximately 10% of CF recovery

204 ators may be beneficial for the treatment of CF patients with PTCs.

205 velopment possibilities for the treatment of CF patients.

206 TEC isolates revealed that 8.6% (n = 13) of "CF negative" (n = 152) and 19.4% (n = 13) of "CF negativ

207 F negative" (n = 152) and 19.4% (n = 13) of "CF negative" LT + STp (n = 67) expressing isolates analy

208 Binding of OligoG CF-5/20 to the bacterial cell surface was demonstrated i

209 e structure of LPS in the presence of OligoG CF-5/20, however, isothermal titration calorimetry demon

210 ion were unaltered in the presence of OligoG CF-5/20.

211 Permeability assays revealed that OligoG CF-5/20 had virtually no membrane-perturbing effects.

212 range of osmolytes when treated with OligoG CF-5/20.

213 Different effects on CF suppression and CT dehalogenation rate were expected

214 o combined properties that favorably opposed CF symptomatology: it reduced inflammation and increased

215 hat Staphylococcus aureus acquisition in our CF population occurred outside the hospital environment

216 for Th1 cells as integrators of perivascular CF and cardiac dysfunction in nonischemic HF.

217 PI-CF subjects had lower acute insulin, C-peptide, and gluc

218 pancreatic-insufficient cystic fibrosis (PI-CF) are at increased risk for developing diabetes.

219 uring glucose potentiation were higher in PI-CF, suggesting impaired proinsulin processing.

220 se tolerance, adolescents and adults with PI-CF have impairments in functional islet mass and associa

221 to the human CF operon CS18 and the porcine CF operon 987P (F6).

222 in plasma from 48 CF patients and in primary CF bronchial epithelial cells (CF-HBEC).

223 ide, and glucagon responses compared with PS-CF and normal control subjects, indicating reduced beta-

224 lular antioxidant properties, especially PSP CF.

225 units of known CFs identified 6 new putative CF variants.

226 used in conjunction with data from a recent CF genome-wide association study (GWAS) meta-analysis to

227 gamma(-/-) Th cells, partially reconstituted CF and HF in TCR-alpha(-/-) recipient mice.

228 l cohort study, was conducted during regular CF clinic visits in the first 12 months of life at 28 US

229 less, detailed reports on lncRNAs regulating CF biology and describing their implication in cardiac r

230 To reproduce CF conditions, reduced chloride secretion, increased pot

231 ation of the presence or absence of PPW, RF, CF, and creases.

232 intermolecular interactions through CF...S, CF...H, and CF...pi noncovalent interactions and enhance

233 ative stabilization through sigmaCC -->sigma*CF interactions are the underlying driving force for the

234 Thirty-six CF patients were followed for a median of 24.5 years (in

235 g them co-fractionation / mass spectrometry (CF-MS), but it remains difficult to identify directly co

236 ator, surgeon, epidemiologist, statistician, CF adult, and a parent.

237 domonas aeruginosa Here, we demonstrate that CF mice are highly susceptible to pulmonary infections w

238 PTEN and the CF transmembrane conductance regulator (CFTR) interacted

239 uld enhance P. aeruginosa clearance from the CF airway by activating PTEN-mediated anti-bacterial res

240 athway contributing to IL-8 secretion in the CF bronchial epithelium with KL functioning as an endocr

241 neutropenia: 74 [17%] of 446 patients in the CF group vs 101 [23%] of 441 people in the ECX group).

242 ere were 21022 individuals (52% male) in the CF patient cohort in 2003 comprised of 12702 children an

243 he development of glucose intolerance in the CF pediatric population, and to CFRD, later in life.

244 The flame in the CF regime exhibits considerable flame-generated enstroph

245 nificantly to the enstrophy transport in the CF regime.

246 In the CF sputum, particle diffusion was spatially heterogeneou

247 this study introduces the development of the CF-MIMS (conception for field experiments, membrane choi

248 also presents two field applications of the CF-MIMS involving the well-logging of dissolved gases an

249 tion of transcription by RNAPII requires the CF IA complex.

250 To address this risk, the CF Foundation convened a multi-stakeholder task force to

251 requency analysis (1 measure every 2 s), the CF-MIMS (Continuous Flow Membrane Inlet Mass Spectromete

252 We hypothesize that the CF suppression seen for amino acids is caused by stabili

253 were recruited and 451 were assigned to the CF group and 446 to the ECX group.

254 onclusion, mucoid Pseudomonas adapted to the CF-lung remained able to invade the allograft.

255 dy from January 2003 to March 2009 using the CF Foundation Patient Registry merged with Centers for D

256 may contribute to its persistence within the CF airways.

257 ablish a base to identify and quantify these CF degradation mechanisms in the field.

258 This CF-ALF induced Psl reduction does not alter initial bact

259 improve intermolecular interactions through CF...S, CF...H, and CF...pi noncovalent interactions and

260 We investigated whether CF-adapted Pseudomonas populations invade the donor micr

261 .001) and in older children (P < 0.001) with CF.

262 sk of colorectal cancer (CRC) in adults with CF is 5-10 times greater compared to the general populat

263 erated nucleotide metabolism associated with CF exacerbations.

264 ied additional genomic sites associated with CF lung disease severity.

265 formed in 97 clinically stable children with CF across the pediatric age range (0.2-21.1 yr).

266 ed about early lung disease in children with CF and discusses the implications for future clinical pr

267 Here we show that ASL pH in children with CF is similar to that of children without CF.

268 for pulmonary exacerbations in children with CF.

269 sease progression in preschool children with CF.

270 n early intervention trials in children with CF.

271 ssolution which was linearly correlated with CF suppression and dehalogenation rate.

272 y epithelial cells from mice and humans with CF.

273 erm clinical trajectory for individuals with CF.

274 ficiency in beta-cell number in infants with CF may contribute to the development of glucose intolera

275 ith correlates of disease among infants with CF who underwent NBS has not been well described.

276 ify the multiple tissue defects in mice with CF as well as in cells from subjects with the p.Phe508de

277 over a prescreening cohort of newborns with CF from 20 years before the contemporary cohort (mean z

278 es in lung disease severity in patients with CF (n = 6365; IL8, P = .001; CXCL1, P = .001), confirmin

279 Materials and Methods Twenty patients with CF and 20 matched healthy volunteers underwent functiona

280 n (<4 years of age), and adult patients with CF and CFRD.

281 QV values of patients with CF correlated with forced vital capacity (r = 0.7; 95% c

282 herapeutic target to treat all patients with CF independent of their underlying CFTR mutation.

283 ctive analyses of samples from patients with CF supported the translational relevance of these precli

284 the entire lungs was lower for patients with CF than for control subjects (mean +/- standard deviatio

285 ower lung halves were lower in patients with CF than in control subjects (right, 0.84 +/- 0.2 vs 1.16

286 ntion of home monitoring among patients with CF was able to detect more exacerbations than usual care

287 One hundred ten consecutive patients with CF were screened between January 2014 and July 2015.

288 monocytes sorted from healthy patients with CF-derived MPs during their differentiation into osteocl

289 culous mycobacteria (NTM) from patients with CF.

290 iated loss of lung function in patients with CF.

291 for the isolation of NTM from patients with CF.

292 rway obstruction were found in patients with CF.

293 ol to guide therapies in young patients with CF.

294 pulmonary exacerbation (PEx) in persons with CF in the United States.

295 Organ transplant recipients with CF should initiate CRC screening at age 30 years within

296 with sweat from individuals with and without CF, demonstrating convenient sweat diagnostics with reli

297 th CF is similar to that of children without CF.

298 Furthermore, young CF tissues displayed significantly smaller insulin-posit

299 The relative number of beta-cells in young CF tissues was reduced by 50% or more when compared to a

300 islet morphology in tissues from very young CF children (<4 years of age), and adult patients with C