Matches in SemOpenAlex for { <https://semopenalex.org/work/W2332428513> ?p ?o ?g. }
- W2332428513 endingPage "579" @default.
- W2332428513 startingPage "572" @default.
- W2332428513 abstract "Abstract We established an efficient strategy to direct human pluripotent stem cells, including human embryonic stem cells (hESCs) and an induced pluripotent stem cell (iPSC) line derived from patients with cystic fibrosis, to differentiate into pancreatic ductal epithelial cells (PDECs). After purification, more than 98% of hESC-derived PDECs expressed functional cystic fibrosis transmembrane conductance regulator (CFTR) protein. In addition, iPSC lines were derived from a patient with CF carrying compound frameshift and mRNA splicing mutations and were differentiated to PDECs. PDECs derived from Weill Cornell cystic fibrosis (WCCF)-iPSCs showed defective expression of mature CFTR protein and impaired chloride ion channel activity, recapitulating functional defects of patients with CF at the cellular level. These studies provide a new methodology to derive pure PDECs expressing CFTR and establish a “disease in a dish” platform to identify drug candidates to rescue the pancreatic defects of patients with CF. Significance An efficient strategy was established to direct human pluripotent stem cells, including human embryonic stem cells (hESCs) and an induced pluripotent stem cell line derived from patients with cystic fibrosis (CF-iPSCs), to differentiate into pancreatic ductal epithelial cells (PDECs). After purification, more than 98% of hESC-PDECs derived from CF-iPSCs showed defective expression of mature cystic fibrosis transmembrane conductance regulator (CFTR) protein and impaired chloride ion channel activity, recapitulating functional pancreatic defects of patients with CF at the cellular level. These studies provide a new methodology for deriving pure PDECs expressing CFTR, and they establish a “disease-in-a-dish” platform for identifying drug candidates to rescue the pancreatic defects of these patients." @default.
- W2332428513 created "2016-06-24" @default.
- W2332428513 creator A5003411069 @default.
- W2332428513 creator A5016189137 @default.
- W2332428513 creator A5024015568 @default.
- W2332428513 creator A5027728578 @default.
- W2332428513 creator A5035133171 @default.
- W2332428513 creator A5053072299 @default.
- W2332428513 creator A5055295478 @default.
- W2332428513 creator A5057038848 @default.
- W2332428513 creator A5058025793 @default.
- W2332428513 creator A5058198258 @default.
- W2332428513 date "2016-03-31" @default.
- W2332428513 modified "2023-10-12" @default.
- W2332428513 title "Modeling Cystic Fibrosis Using Pluripotent Stem Cell-Derived Human Pancreatic Ductal Epithelial Cells" @default.
- W2332428513 cites W1558481665 @default.
- W2332428513 cites W1949252567 @default.
- W2332428513 cites W1971149849 @default.
- W2332428513 cites W1986790770 @default.
- W2332428513 cites W1991013623 @default.
- W2332428513 cites W2003126320 @default.
- W2332428513 cites W2007914388 @default.
- W2332428513 cites W2025724973 @default.
- W2332428513 cites W2034911447 @default.
- W2332428513 cites W2036774130 @default.
- W2332428513 cites W2038815755 @default.
- W2332428513 cites W2040111212 @default.
- W2332428513 cites W2049888495 @default.
- W2332428513 cites W2055710769 @default.
- W2332428513 cites W2076018642 @default.
- W2332428513 cites W2076312582 @default.
- W2332428513 cites W2087326716 @default.
- W2332428513 cites W2088714934 @default.
- W2332428513 cites W2102424187 @default.
- W2332428513 cites W2108160744 @default.
- W2332428513 cites W2110491077 @default.
- W2332428513 cites W2112055817 @default.
- W2332428513 cites W2169102080 @default.
- W2332428513 cites W2169191544 @default.
- W2332428513 cites W2170409651 @default.
- W2332428513 cites W2201543151 @default.
- W2332428513 cites W2220809577 @default.
- W2332428513 doi "https://doi.org/10.5966/sctm.2015-0276" @default.
- W2332428513 hasPubMedCentralId "https://www.ncbi.nlm.nih.gov/pmc/articles/4835252" @default.
- W2332428513 hasPubMedId "https://pubmed.ncbi.nlm.nih.gov/27034411" @default.
- W2332428513 hasPublicationYear "2016" @default.
- W2332428513 type Work @default.
- W2332428513 sameAs 2332428513 @default.
- W2332428513 citedByCount "47" @default.
- W2332428513 countsByYear W23324285132016 @default.
- W2332428513 countsByYear W23324285132017 @default.
- W2332428513 countsByYear W23324285132018 @default.
- W2332428513 countsByYear W23324285132019 @default.
- W2332428513 countsByYear W23324285132020 @default.
- W2332428513 countsByYear W23324285132021 @default.
- W2332428513 countsByYear W23324285132022 @default.
- W2332428513 countsByYear W23324285132023 @default.
- W2332428513 crossrefType "journal-article" @default.
- W2332428513 hasAuthorship W2332428513A5003411069 @default.
- W2332428513 hasAuthorship W2332428513A5016189137 @default.
- W2332428513 hasAuthorship W2332428513A5024015568 @default.
- W2332428513 hasAuthorship W2332428513A5027728578 @default.
- W2332428513 hasAuthorship W2332428513A5035133171 @default.
- W2332428513 hasAuthorship W2332428513A5053072299 @default.
- W2332428513 hasAuthorship W2332428513A5055295478 @default.
- W2332428513 hasAuthorship W2332428513A5057038848 @default.
- W2332428513 hasAuthorship W2332428513A5058025793 @default.
- W2332428513 hasAuthorship W2332428513A5058198258 @default.
- W2332428513 hasBestOaLocation W23324285131 @default.
- W2332428513 hasConcept C104317684 @default.
- W2332428513 hasConcept C107459253 @default.
- W2332428513 hasConcept C134018914 @default.
- W2332428513 hasConcept C145103041 @default.
- W2332428513 hasConcept C145822097 @default.
- W2332428513 hasConcept C148738053 @default.
- W2332428513 hasConcept C2776938444 @default.
- W2332428513 hasConcept C2778148616 @default.
- W2332428513 hasConcept C2778428886 @default.
- W2332428513 hasConcept C2778764654 @default.
- W2332428513 hasConcept C2779389132 @default.
- W2332428513 hasConcept C28328180 @default.
- W2332428513 hasConcept C502942594 @default.
- W2332428513 hasConcept C54355233 @default.
- W2332428513 hasConcept C86803240 @default.
- W2332428513 hasConcept C95444343 @default.
- W2332428513 hasConceptScore W2332428513C104317684 @default.
- W2332428513 hasConceptScore W2332428513C107459253 @default.
- W2332428513 hasConceptScore W2332428513C134018914 @default.
- W2332428513 hasConceptScore W2332428513C145103041 @default.
- W2332428513 hasConceptScore W2332428513C145822097 @default.
- W2332428513 hasConceptScore W2332428513C148738053 @default.
- W2332428513 hasConceptScore W2332428513C2776938444 @default.
- W2332428513 hasConceptScore W2332428513C2778148616 @default.
- W2332428513 hasConceptScore W2332428513C2778428886 @default.
- W2332428513 hasConceptScore W2332428513C2778764654 @default.
- W2332428513 hasConceptScore W2332428513C2779389132 @default.
- W2332428513 hasConceptScore W2332428513C28328180 @default.
- W2332428513 hasConceptScore W2332428513C502942594 @default.