相关参考文献
注意:仅列出部分参考文献,下载原文获取全部文献信息。Two Small Molecules Restore Stability to a Subpopulation of the Cystic Fibrosis Transmembrane Conductance Regulator with the Predominant Disease-causing Mutation
Xin Meng et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2017)
Pharmacological Rescue of Conditionally Reprogrammed Cystic Fibrosis Bronchial Epithelial Cells
Martina Gentzsch et al.
AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY (2017)
Combination therapy with cystic fibrosis transmembrane conductance regulator modulators augment the airway functional microanatomy
Susan E. Birket et al.
AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY (2016)
Airway Progenitor Clone Formation Is Enhanced by Y-27632-Dependent Changes in the Transcriptome
Susan D. Reynolds et al.
AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY (2016)
Using Drugs to Probe the Variability of Trans-Epithelial Airway Resistance
Kendra Tosoni et al.
PLOS ONE (2016)
Characterizing responses to CFTR-modulating drugs using rectal organoids derived from subjects with cystic fibrosis
Johanna F. Dekkers et al.
SCIENCE TRANSLATIONAL MEDICINE (2016)
Clinical expression of patients with the D1152H CFTR mutation
Vito Terlizzi et al.
JOURNAL OF CYSTIC FIBROSIS (2015)
Lumacaftor-Ivacaftor in Patients with Cystic Fibrosis Homozygous for Phe508del CFTR
C. E. Wainwright et al.
NEW ENGLAND JOURNAL OF MEDICINE (2015)
Measurements of Functional Responses in Human Primary Lung Cells as a Basis for Personalized Therapy for Cystic Fibrosis
Nikhil T. Awatade et al.
EBIOMEDICINE (2015)
Cystic fibrosis genetics: from molecular understanding to clinical application
Garry R. Cutting
NATURE REVIEWS GENETICS (2015)
Potentiators of Defective ΔF508-CFTR Gating that Do Not Interfere with Corrector Action
Puay-Wah Phuan et al.
MOLECULAR PHARMACOLOGY (2015)
Partial inhibition of Cdk1 in G2 phase overrides the SAC and decouples mitotic events
Rachael A. McCloy et al.
CELL CYCLE (2014)
Biosynthesis of cystic fibrosis transmembrane conductance regulator
Iwona M. Pranke et al.
INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY (2014)
Sweat chloride as a biomarker of CFTR activity: Proof of concept and ivacaftor clinical trial data
Frank J. Accurso et al.
JOURNAL OF CYSTIC FIBROSIS (2014)
Effect of ivacaftor on CFTR forms with missense mutations associated with defects in protein processing or function
Fredrick Van Goor et al.
JOURNAL OF CYSTIC FIBROSIS (2014)
Efficacy and safety of ivacaftor in patients with cystic fibrosis and a non-G551D gating mutation
Kris De Boeck et al.
JOURNAL OF CYSTIC FIBROSIS (2014)
Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression
Guido Veit et al.
SCIENCE TRANSLATIONAL MEDICINE (2014)
Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis
Deborah M. Cholon et al.
SCIENCE TRANSLATIONAL MEDICINE (2014)
Development of primary human nasal epithelial cell cultures for the study of cystic fibrosis pathophysiology
F. de Courcey et al.
AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY (2012)
Conditionally reprogrammed cells represent a stem-like state of adult epithelial cells
Frank A. Suprynowicz et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2012)
Assessing the Disease-Liability of Mutations in CFTR
Claude Ferec et al.
COLD SPRING HARBOR PERSPECTIVES IN MEDICINE (2012)
Human cystic fibrosis airway epithelia have reduced Cl- conductance but not increased Na+ conductance
Omar A. Itani et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2011)
Correction of the F508del-CFTR protein processing defect in vitro by the investigational drug VX-809
Fredrick Van Goor et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2011)
Clinical Phenotype and Genotype of Children with Borderline Sweat Test and Abnormal Nasal Epithelial Chloride Transport
Isabelle Sermet-Gaudelus et al.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2010)
A new complex allele of the CFTR gene partially explains the variable phenotype of the L997F mutation
Marco Lucarelli et al.
GENETICS IN MEDICINE (2010)
Effect of VX-770 in Persons with Cystic Fibrosis and the G551D-CFTR Mutation
Frank J. Accurso et al.
NEW ENGLAND JOURNAL OF MEDICINE (2010)
Mutations that permit residual CFTR function delay acquisition of multiple respiratory pathogens in CF patients
Deanna M. Green et al.
RESPIRATORY RESEARCH (2010)
Rescue of CF airway epithelial cell function in vitro by a CFTR potentiator, VX-770
Fredrick Van Goor et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2009)
Nasal Epithelial Cells as Surrogates for Bronchial Epithelial Cells in Airway Inflammation Studies
Catherine M. McDougall et al.
AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY (2008)
Feasibility of nasal epithelial brushing for the study of airway epithelial functions in CF infants
Katharina Mosler et al.
JOURNAL OF CYSTIC FIBROSIS (2008)
Upregulated expression of ENaC in human CF nasal epithelium
Nadine Bangel et al.
JOURNAL OF CYSTIC FIBROSIS (2008)
Basolateral Cl channels in primary airway epithelial cultures
Horst Fischer et al.
AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY (2007)
Rescue of ΔF508-CFTR trafficking and gating in human cystic fibrosis airway primary cultures by small molecules
F Van Goor et al.
AMERICAN JOURNAL OF PHYSIOLOGY-LUNG CELLULAR AND MOLECULAR PHYSIOLOGY (2006)
Long-term cultures of polarized airway epithelial cells from patients with cystic fibrosis
L Wiszniewski et al.
AMERICAN JOURNAL OF RESPIRATORY CELL AND MOLECULAR BIOLOGY (2006)
Expression of alpha-, beta-, and gamma-hENaC mRNA in the human nasal, bronchial, and distal lung epithelium
OM Pitkanen et al.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2001)