Published in J Physiol on February 09, 2016
George Ralph Mines (1886-1914): the dawn of cardiac nonlinear dynamics. J Physiol (2016) 0.75
Inhibitory effects and mechanism of dihydroberberine on hERG channels expressed in HEK293 cells. PLoS One (2017) 0.75
Drug-induced prolongation of the QT interval. N Engl J Med (2004) 10.96
Molecular chaperones in protein folding and proteostasis. Nature (2011) 8.63
HERG, a human inward rectifier in the voltage-gated potassium channel family. Science (1995) 7.26
Structure of TPR domain-peptide complexes: critical elements in the assembly of the Hsp70-Hsp90 multichaperone machine. Cell (2000) 7.25
HSP90 at the hub of protein homeostasis: emerging mechanistic insights. Nat Rev Mol Cell Biol (2010) 7.16
hERG potassium channels and cardiac arrhythmia. Nature (2006) 6.63
Pathways of chaperone-mediated protein folding in the cytosol. Nat Rev Mol Cell Biol (2004) 6.45
The co-chaperone CHIP regulates protein triage decisions mediated by heat-shock proteins. Nat Cell Biol (2001) 6.10
Structure and mechanism of the Hsp90 molecular chaperone machinery. Annu Rev Biochem (2006) 5.82
Pathways and mechanisms of endocytic recycling. Nat Rev Mol Cell Biol (2009) 5.60
Molecular and cellular mechanisms of cardiac arrhythmias. Cell (2001) 5.41
The Hsc70 co-chaperone CHIP targets immature CFTR for proteasomal degradation. Nat Cell Biol (2001) 5.36
Hsp90 cochaperone Aha1 downregulation rescues misfolding of CFTR in cystic fibrosis. Cell (2006) 5.01
Molecular chaperones Hsp90 and Hsp70 deliver preproteins to the mitochondrial import receptor Tom70. Cell (2003) 4.46
Prevalence of the congenital long-QT syndrome. Circulation (2009) 4.32
A structural basis for drug-induced long QT syndrome. Proc Natl Acad Sci U S A (2000) 4.06
Large-scale prediction and testing of drug activity on side-effect targets. Nature (2012) 3.88
Properties of HERG channels stably expressed in HEK 293 cells studied at physiological temperature. Biophys J (1998) 3.83
Sequential quality-control checkpoints triage misfolded cystic fibrosis transmembrane conductance regulator. Cell (2006) 3.73
Quantitative analysis of HSP90-client interactions reveals principles of substrate recognition. Cell (2012) 3.44
Most LQT2 mutations reduce Kv11.1 (hERG) current by a class 2 (trafficking-deficient) mechanism. Circulation (2006) 3.23
Crystal structure and functional analysis of the HERG potassium channel N terminus: a eukaryotic PAS domain. Cell (1998) 3.22
CHIP is a chaperone-dependent E3 ligase that ubiquitylates unfolded protein. EMBO Rep (2001) 3.19
Road to ruin: targeting proteins for degradation in the endoplasmic reticulum. Science (2011) 3.19
Peripheral protein quality control removes unfolded CFTR from the plasma membrane. Science (2010) 2.92
Role of the cytosolic chaperones Hsp70 and Hsp90 in maturation of the cardiac potassium channel HERG. Circ Res (2003) 2.27
hERG K(+) channels: structure, function, and clinical significance. Physiol Rev (2012) 2.24
Discovery of a small molecule activator of the human ether-a-go-go-related gene (HERG) cardiac K+ channel. Mol Pharmacol (2004) 2.15
Gp78 cooperates with RMA1 in endoplasmic reticulum-associated degradation of CFTRDeltaF508. Mol Biol Cell (2008) 2.14
The Hsp90 chaperone machinery: conformational dynamics and regulation by co-chaperones. Biochim Biophys Acta (2011) 1.90
Molecular determinant of high-affinity dofetilide binding to HERG1 expressed in Xenopus oocytes: involvement of S6 sites. Mol Pharmacol (2000) 1.89
Structure, function and regulation of the hsp90 machinery. Biomed J (2013) 1.81
Pentamidine-induced long QT syndrome and block of hERG trafficking. J Pharmacol Exp Ther (2004) 1.76
Correction of both NBD1 energetics and domain interface is required to restore ΔF508 CFTR folding and function. Cell (2012) 1.73
A foldable CFTR{Delta}F508 biogenic intermediate accumulates upon inhibition of the Hsc70-CHIP E3 ubiquitin ligase. J Cell Biol (2004) 1.70
Derlin-1 promotes the efficient degradation of the cystic fibrosis transmembrane conductance regulator (CFTR) and CFTR folding mutants. J Biol Chem (2006) 1.69
Drug-induced long QT syndrome: hERG K+ channel block and disruption of protein trafficking by fluoxetine and norfluoxetine. Br J Pharmacol (2006) 1.68
Potentiator ivacaftor abrogates pharmacological correction of ΔF508 CFTR in cystic fibrosis. Sci Transl Med (2014) 1.67
Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression. Sci Transl Med (2014) 1.64
An analysis of the attrition of drug candidates from four major pharmaceutical companies. Nat Rev Drug Discov (2015) 1.62
The binding site for channel blockers that rescue misprocessed human long QT syndrome type 2 ether-a-gogo-related gene (HERG) mutations. J Biol Chem (2001) 1.60
Physicochemical features of the HERG channel drug binding site. J Biol Chem (2003) 1.54
Clathrin-independent endocytosis: mechanisms and function. Curr Opin Cell Biol (2011) 1.53
BAG-2 acts as an inhibitor of the chaperone-associated ubiquitin ligase CHIP. Mol Biol Cell (2005) 1.52
Hsp70 chaperone dynamics and molecular mechanism. Trends Biochem Sci (2013) 1.49
Nonsense mutations in hERG cause a decrease in mutant mRNA transcripts by nonsense-mediated mRNA decay in human long-QT syndrome. Circulation (2007) 1.43
Mechanism of action of a novel human ether-a-go-go-related gene channel activator. Mol Pharmacol (2005) 1.43
Differential effects of Hsc70 and Hsp70 on the intracellular trafficking and functional expression of epithelial sodium channels. Proc Natl Acad Sci U S A (2006) 1.39
Role of glycosylation in cell surface expression and stability of HERG potassium channels. Am J Physiol Heart Circ Physiol (2002) 1.38
Extracellular K+ concentration controls cell surface density of IKr in rabbit hearts and of the HERG channel in human cell lines. J Clin Invest (2009) 1.37
Plasticity of polyubiquitin recognition as lysosomal targeting signals by the endosomal sorting machinery. Mol Biol Cell (2007) 1.37
Molecular determinants of voltage-dependent human ether-a-go-go related gene (HERG) K+ channel block. J Biol Chem (2002) 1.33
Structural basis of action for a human ether-a-go-go-related gene 1 potassium channel activator. Proc Natl Acad Sci U S A (2007) 1.32
Cooperative assembly and misfolding of CFTR domains in vivo. Mol Biol Cell (2009) 1.31
Co-chaperone FKBP38 promotes HERG trafficking. J Biol Chem (2007) 1.30
Identification of the cyclic-nucleotide-binding domain as a conserved determinant of ion-channel cell-surface localization. J Cell Sci (2005) 1.25
Functional divergence between co-chaperones of Hsc70. J Biol Chem (2008) 1.17
Finding the will and the way of ERAD substrate retrotranslocation. Curr Opin Cell Biol (2012) 1.16
N-linked glycosylation sites determine HERG channel surface membrane expression. J Physiol (1999) 1.16
The HERG K+ channel: progress in understanding the molecular basis of its unusual gating kinetics. Eur Biophys J (2003) 1.11
Ubiquitin-dependent sorting in endocytosis. Cold Spring Harb Perspect Biol (2014) 1.10
Hsp40 chaperones promote degradation of the HERG potassium channel. J Biol Chem (2009) 1.09
Coexistence of hERG current block and disruption of protein trafficking in ketoconazole-induced long QT syndrome. Br J Pharmacol (2007) 1.09
Structural basis of nucleotide exchange and client binding by the Hsp70 cochaperone Bag2. Nat Struct Mol Biol (2008) 1.08
Characterization of hERG1a and hERG1b potassium channels-a possible role for hERG1b in the I (Kr) current. Pflugers Arch (2008) 1.07
Cardiac glycosides as novel inhibitors of human ether-a-go-go-related gene channel trafficking. J Pharmacol Exp Ther (2006) 1.07
hERG K+ channel-associated cardiac effects of the antidepressant drug desipramine. Naunyn Schmiedebergs Arch Pharmacol (2010) 1.02
Binding of human nucleotide exchange factors to heat shock protein 70 (Hsp70) generates functionally distinct complexes in vitro. J Biol Chem (2013) 1.01
Endoplasmic reticulum retention and rescue by heteromeric assembly regulate human ERG 1a/1b surface channel composition. J Biol Chem (2007) 0.98
The serum- and glucocorticoid-inducible kinases SGK1 and SGK3 regulate hERG channel expression via ubiquitin ligase Nedd4-2 and GTPase Rab11. J Biol Chem (2013) 0.97
Quality control for unfolded proteins at the plasma membrane. J Cell Biol (2010) 0.96
The role of monoubiquitination in endocytic degradation of human ether-a-go-go-related gene (hERG) channels under low K+ conditions. J Biol Chem (2010) 0.96
Changes in channel trafficking and protein stability caused by LQT2 mutations in the PAS domain of the HERG channel. PLoS One (2012) 0.94
Reciprocal control of hERG stability by Hsp70 and Hsc70 with implication for restoration of LQT2 mutant stability. Circ Res (2010) 0.92
Cell surface expression of human ether-a-go-go-related gene (hERG) channels is regulated by caveolin-3 protein via the ubiquitin ligase Nedd4-2. J Biol Chem (2012) 0.92
Large-scale mutational analysis of Kv11.1 reveals molecular insights into type 2 long QT syndrome. Nat Commun (2014) 0.92
Domain:domain interactions within Hop, the Hsp70/Hsp90 organizing protein, are required for protein stability and structure. Protein Sci (2006) 0.92
Human heat shock protein 105/110 kDa (Hsp105/110) regulates biogenesis and quality control of misfolded cystic fibrosis transmembrane conductance regulator at multiple levels. J Biol Chem (2012) 0.92
Antidepressant-induced ubiquitination and degradation of the cardiac potassium channel hERG. J Biol Chem (2011) 0.90
Inhibition of nonsense-mediated mRNA decay by antisense morpholino oligonucleotides restores functional expression of hERG nonsense and frameshift mutations in long-QT syndrome. J Mol Cell Cardiol (2010) 0.90
hERG 1b is critical for human cardiac repolarization. Proc Natl Acad Sci U S A (2014) 0.90
The hERG channel is dependent upon the Hsp90α isoform for maturation and trafficking. Mol Pharm (2012) 0.89
The DNAJA2 substrate release mechanism is essential for chaperone-mediated folding. J Biol Chem (2012) 0.87
The role of the cytosolic HSP70 chaperone system in diseases caused by misfolding and aberrant trafficking of ion channels. Dis Model Mech (2014) 0.86
Molecular determinants of human ether-à-go-go-related gene 1 (hERG1) K+ channel activation by NS1643. Mol Pharmacol (2010) 0.85
Hsp90 prevents interaction between CHIP and HERG proteins to facilitate maturation of wild-type and mutant HERG proteins. Cardiovasc Res (2013) 0.85
Ubiquitination-dependent quality control of hERG K+ channel with acquired and inherited conformational defect at the plasma membrane. Mol Biol Cell (2013) 0.85
Trafficking defects in PAS domain mutant Kv11.1 channels: roles of reduced domain stability and altered domain-domain interactions. Biochem J (2013) 0.84
Role of the cytoplasmic N-terminal Cap and Per-Arnt-Sim (PAS) domain in trafficking and stabilization of Kv11.1 channels. J Biol Chem (2014) 0.80
Rab11-dependent Recycling of the Human Ether-a-go-go-related Gene (hERG) Channel. J Biol Chem (2015) 0.77
Endocytosis of HERG is clathrin-independent and involves arf6. PLoS One (2013) 0.77