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Programme in Molecular Structure and Function
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Studies of the molecular basis for cystic fibrosis using purified reconstituted CFTR protein.
Methods in molecular medicine , 2002 | Pubmed ID: 11917519
CFTR directly mediates nucleotide-regulated glutathione flux.
The EMBO journal May, 2003 | Pubmed ID: 12727866
Stable dimeric assembly of the second membrane-spanning domain of CFTR (cystic fibrosis transmembrane conductance regulator) reconstitutes a chloride-selective pore.
The Biochemical journal Nov, 2003 | Pubmed ID: 12892562
Phosphorylation-induced conformational changes of cystic fibrosis transmembrane conductance regulator monitored by attenuated total reflection-Fourier transform IR spectroscopy and fluorescence spectroscopy.
The Journal of biological chemistry Feb, 2004 | Pubmed ID: 14660584
Methods to study CFTR protein in vitro.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society Aug, 2004 | Pubmed ID: 15463933
The patch-clamp and planar lipid bilayer techniques: powerful and versatile tools to investigate the CFTR Cl- channel.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society Aug, 2004 | Pubmed ID: 15463939
Determination of CFTR chloride channel activity and pharmacology using radiotracer flux methods.
Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society Aug, 2004 | Pubmed ID: 15463942
Evaluation of the membrane-spanning domain of ClC-2.
The Biochemical journal Jun, 2006 | Pubmed ID: 16526942
Nucleotides bind to the C-terminus of ClC-5.
The Biochemical journal Sep, 2006 | Pubmed ID: 16686597
The intact CFTR protein mediates ATPase rather than adenylate kinase activity.
The Biochemical journal Jun, 2008 | Pubmed ID: 18241200
Probing structure-function relationships and gating mechanisms in the CorA Mg2+ transport system.
The Journal of biological chemistry Apr, 2008 | Pubmed ID: 18276588
Evidence for a superoxide permeability pathway in endosomal membranes.
Molecular and cellular biology Jun, 2008 | Pubmed ID: 18378695
Direct interaction of a small-molecule modulator with G551D-CFTR, a cystic fibrosis-causing mutation associated with severe disease.
The Biochemical journal Feb, 2009 | Pubmed ID: 18945216
A small-molecule modulator interacts directly with deltaPhe508-CFTR to modify its ATPase activity and conformational stability.
Molecular pharmacology Jun, 2009 | Pubmed ID: 19339490
Structural basis for alginate secretion across the bacterial outer membrane.
Proceedings of the National Academy of Sciences of the United States of America Aug, 2011 | Pubmed ID: 21778407
Synthesis and properties of molecular probes for the rescue site on mutant cystic fibrosis transmembrane conductance regulator.
Journal of medicinal chemistry Dec, 2011 | Pubmed ID: 22074181
Cystic fibrosis transmembrane conductance regulator (CFTR) potentiator VX-770 (ivacaftor) opens the defective channel gate of mutant CFTR in a phosphorylation-dependent but ATP-independent manner.
The Journal of biological chemistry Oct, 2012 | Pubmed ID: 22942289
VX-809 and related corrector compounds exhibit secondary activity stabilizing active F508del-CFTR after its partial rescue to the cell surface.
Chemistry & biology May, 2014 | Pubmed ID: 24726831
Hospital for Sick Children
Paul D. W. Eckford1,
Canhui Li1,
Christine E. Bear1,2,3
1Programme in Molecular Structure and Function, Hospital for Sick Children,
2Department of Biochemistry, University of Toronto,
3Department of Physiology, University of Toronto
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