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。Repairing the basic defect in cystic fibrosis - one approach is not enough
Carlos M. Farinha et al.
FEBS JOURNAL (2016)
Capturing the Direct Binding of CFTR Correctors to CFTR by Using Click Chemistry
Chandrima Sinha et al.
CHEMBIOCHEM (2015)
Rescuing Trafficking Mutants of the ATP-binding Cassette Protein, ABCA4, with Small Molecule Correctors as a Treatment for Stargardt Eye Disease
Inna Sabirzhanova et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2015)
Combination of Correctors Rescue ΔF508-CFTR by Reducing Its Association with Hsp40 and Hsp27
Miqueias Lopes-Pacheco et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2015)
Correcting the Cystic Fibrosis Disease Mutant, A455E CFTR
Liudmila Cebotaru et al.
PLOS ONE (2014)
Modulation of the Maladaptive Stress Response to Manage Diseases of Protein Folding
Daniela Martino Roth et al.
PLOS BIOLOGY (2014)
A CFTR corrector (lumacaftor) and a CFTR potentiator (ivacaftor) for treatment of patients with cystic fibrosis who have a phe508del CFTR mutation: a phase 2 randomised controlled trial
Michael P. Boyle et al.
LANCET RESPIRATORY MEDICINE (2014)
Small heat shock proteins target mutant cystic fibrosis transmembrane conductance regulator for degradation via a small ubiquitin-like modifier-dependent pathway
Annette Ahner et al.
MOLECULAR BIOLOGY OF THE CELL (2013)
Mechanism-based corrector combination restores ΔF508-CFTR folding and function
Tsukasa Okiyoneda et al.
NATURE CHEMICAL BIOLOGY (2013)
Defining the disease liability of variants in the cystic fibrosis transmembrane conductance regulator gene
Patrick R. Sosnay et al.
NATURE GENETICS (2013)
The Hsp90 chaperone machinery: Conformational dynamics and regulation by co-chaperones
Jing Li et al.
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH (2012)
Ivacaftor in Subjects With Cystic Fibrosis Who Are Homozygous for the F508del-CFTR Mutation
Patrick A. Flume et al.
CHEST (2012)
Results of a phase IIa study of VX-809, an investigational CFTR corrector compound, in subjects with cystic fibrosis homozygous for the F508del-CFTR mutation
J. P. Clancy et al.
THORAX (2012)
Managing Cystic Fibrosis Strategies That Increase Life Expectancy and Improve Quality of Life
Malena Cohen-Cymberknoh et al.
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2011)
The endoplasmic reticulum-associated Hsp40 DNAJB12 and Hsc70 cooperate to facilitate RMA1 E3-dependent degradation of nascent CFTRΔF508
Diane E. Grove et al.
MOLECULAR BIOLOGY OF THE CELL (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)
Emergent Properties of Proteostasis in Managing Cystic Fibrosis
William E. Balch et al.
COLD SPRING HARBOR PERSPECTIVES IN BIOLOGY (2011)
Reduced histone deacetylase 7 activity restores function to misfolded CFTR in cystic fibrosis
Darren M. Hutt et al.
NATURE CHEMICAL BIOLOGY (2010)
The Primary Folding Defect and Rescue of ΔF508 CFTR Emerge during Translation of the Mutant Domain
Hanneke Hoelen et al.
PLOS ONE (2010)
Peripheral Protein Quality Control Removes Unfolded CFTR from the Plasma Membrane
Tsukasa Okiyoneda et al.
SCIENCE (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)
Phenylalanine-508 mediates a cytoplasmic-membrane domain contact in the CFTR 3D structure crucial to assembly and channel function
Adrian W. R. Serohijos et al.
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA (2008)
Correctors promote maturation of cystic fibrosis transmembrane conductance regulator (CFTR)-processing mutants by binding to the protein
Ying Wang et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2007)
HDAC6-p97/VCP controlled polyubiquitin chain turnover
Cyril Boyault et al.
EMBO JOURNAL (2006)
Selective inhibition of endoplasmic reticulum-associated degradation rescues ΔF508-cystic fibrosis transmembrane regulator and suppresses interleukin-8 levels
Neeraj Vij et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2006)
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)
The short apical membrane half-life of rescued ΔF508-cystic fibrosis transmembrane conductance regulator (CFTR) results from accelerated endocytosis of ΔF508-CFTR in polarized human airway epithelial cells
A Swiatecka-Urban et al.
JOURNAL OF BIOLOGICAL CHEMISTRY (2005)
Small-molecule correctors of defective ΔF508-CFTR cellular processing identified by high-throughput screening
N Pedemonte et al.
JOURNAL OF CLINICAL INVESTIGATION (2005)
Misfolding diverts CFTR from recycling to degradation: quality control at early endosomes
M Sharma et al.
JOURNAL OF CELL BIOLOGY (2004)
Structure of nucleotide-binding domain 1 of the cystic fibrosis transmembrane conductance regulator
HA Lewis et al.
EMBO JOURNAL (2004)
CFTR and chaperones - Processing and degradation
MD Amaral
JOURNAL OF MOLECULAR NEUROSCIENCE (2004)
The deacetylase HDAC6 regulates aggresome formation and cell viability in response to misfolded protein stress
Y Kawaguchi et al.
CELL (2003)
Lung infections associated with cystic fibrosis
JB Lyczak et al.
CLINICAL MICROBIOLOGY REVIEWS (2002)
分享论文
