Published in J Biol Chem on November 25, 2009
The delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiology. Physiol Rev (2012) 1.95
Cysteine string protein limits expression of the large conductance, calcium-activated K⁺ (BK) channel. PLoS One (2014) 1.46
Formation of chimeric genes by copy-number variation as a mutational mechanism in schizophrenia. Am J Hum Genet (2013) 1.10
Identification of human Ether-à-go-go related gene modulators by three screening platforms in an academic drug-discovery setting. Assay Drug Dev Technol (2010) 1.07
CHIP promotes human telomerase reverse transcriptase degradation and negatively regulates telomerase activity. J Biol Chem (2010) 0.93
Large-scale mutational analysis of Kv11.1 reveals molecular insights into type 2 long QT syndrome. Nat Commun (2014) 0.92
Translational toxicology and rescue strategies of the hERG channel dysfunction: biochemical and molecular mechanistic aspects. Acta Pharmacol Sin (2014) 0.88
The DNAJA2 substrate release mechanism is essential for chaperone-mediated folding. J Biol Chem (2012) 0.87
The large conductance, calcium-activated K+ (BK) channel is regulated by cysteine string protein. Sci Rep (2013) 0.87
FKBP38 peptidylprolyl isomerase promotes the folding of cystic fibrosis transmembrane conductance regulator in the endoplasmic reticulum. J Biol Chem (2011) 0.86
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
Optimal functional levels of activation-induced deaminase specifically require the Hsp40 DnaJa1. EMBO J (2011) 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 defect and proteasomal degradation contribute to the phenotype of a novel KCNH2 long QT syndrome mutation. PLoS One (2011) 0.85
Molecular Pathophysiology of Congenital Long QT Syndrome. Physiol Rev (2017) 0.85
Co-chaperone HSJ1a dually regulates the proteasomal degradation of ataxin-3. PLoS One (2011) 0.84
Identification of GABA(C) receptor protein homeostasis network components from three tandem mass spectrometry proteomics approaches. J Proteome Res (2013) 0.80
DnaJA1/Hsp40 is co-opted by influenza A virus to enhance its viral RNA polymerase activity. J Virol (2014) 0.79
Mechanisms underlying the protein-kinase mediated regulation of the HERG potassium channel synthesis. Biochim Biophys Acta (2012) 0.79
Emerging concepts in the pharmacogenomics of arrhythmias: ion channel trafficking. Expert Rev Cardiovasc Ther (2010) 0.78
Sequence and structure-specific elements of HERG mRNA determine channel synthesis and trafficking efficiency. FASEB J (2013) 0.77
Mechanistic basis for type 2 long QT syndrome caused by KCNH2 mutations that disrupt conserved arginine residues in the voltage sensor. J Membr Biol (2013) 0.76
Schizophrenia-Associated hERG channel Kv11.1-3.1 Exhibits a Unique Trafficking Deficit that is Rescued Through Proteasome Inhibition for High Throughput Screening. Sci Rep (2016) 0.76
MiR-17-5p impairs trafficking of H-ERG K+ channel protein by targeting multiple er stress-related chaperones during chronic oxidative stress. PLoS One (2013) 0.76
hERG quality control and the long QT syndrome. J Physiol (2016) 0.76
Transcriptional Network Architecture of Breast Cancer Molecular Subtypes. Front Physiol (2016) 0.75
RING finger protein RNF207, a novel regulator of cardiac excitation. J Biol Chem (2014) 0.75
Bag1 Co-chaperone Promotes TRC8 E3 Ligase-dependent Degradation of Misfolded Human Ether a Go-Go-related Gene (hERG) Potassium Channels. J Biol Chem (2016) 0.75
Molecular pathogenesis of long QT syndrome type 2. J Arrhythm (2016) 0.75
A mechanistic link between an inherited and an acquired cardiac arrhythmia: HERG encodes the IKr potassium channel. Cell (1995) 11.62
A molecular basis for cardiac arrhythmia: HERG mutations cause long QT syndrome. Cell (1995) 10.18
Hsp70 chaperones: cellular functions and molecular mechanism. Cell Mol Life Sci (2005) 9.75
HL-1 cells: a cardiac muscle cell line that contracts and retains phenotypic characteristics of the adult cardiomyocyte. Proc Natl Acad Sci U S A (1998) 8.22
Regulation of signaling protein function and trafficking by the hsp90/hsp70-based chaperone machinery. Exp Biol Med (Maywood) (2003) 8.04
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
Pathways of chaperone-mediated protein folding in the cytosol. Nat Rev Mol Cell Biol (2004) 6.45
Structure and mechanism of the Hsp90 molecular chaperone machinery. Annu Rev Biochem (2006) 5.82
A family of potassium channel genes related to eag in Drosophila and mammals. Proc Natl Acad Sci U S A (1994) 5.77
Posttranslational quality control: folding, refolding, and degrading proteins. Science (1999) 5.55
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
Sequential quality-control checkpoints triage misfolded cystic fibrosis transmembrane conductance regulator. Cell (2006) 3.73
CFTR function and prospects for therapy. Annu Rev Biochem (2008) 3.71
DnaJ-like proteins: molecular chaperones and specific regulators of Hsp70. Trends Biochem Sci (1994) 3.65
The diversity of the DnaJ/Hsp40 family, the crucial partners for Hsp70 chaperones. Cell Mol Life Sci (2006) 3.62
Most LQT2 mutations reduce Kv11.1 (hERG) current by a class 2 (trafficking-deficient) mechanism. Circulation (2006) 3.23
Cooperation of a ubiquitin domain protein and an E3 ubiquitin ligase during chaperone/proteasome coupling. Curr Biol (2001) 3.21
CHIP: a link between the chaperone and proteasome systems. Cell Stress Chaperones (2003) 2.98
Cardiac physiology at the cellular level: use of cultured HL-1 cardiomyocytes for studies of cardiac muscle cell structure and function. Am J Physiol Heart Circ Physiol (2004) 2.84
Misfolding diverts CFTR from recycling to degradation: quality control at early endosomes. J Cell Biol (2004) 2.74
The Hdj-2/Hsc70 chaperone pair facilitates early steps in CFTR biogenesis. EMBO J (1999) 2.67
The ubiquitin-related BAG-1 provides a link between the molecular chaperones Hsc70/Hsp70 and the proteasome. J Biol Chem (2000) 2.66
Spectrum of HERG K+-channel dysfunction in an inherited cardiac arrhythmia. Proc Natl Acad Sci U S A (1996) 2.63
From the cradle to the grave: molecular chaperones that may choose between folding and degradation. EMBO Rep (2001) 2.55
HERG channel dysfunction in human long QT syndrome. Intracellular transport and functional defects. J Biol Chem (1998) 2.52
Role of the cytosolic chaperones Hsp70 and Hsp90 in maturation of the cardiac potassium channel HERG. Circ Res (2003) 2.27
Polyglutamine-expanded androgen receptors form aggregates that sequester heat shock proteins, proteasome components and SRC-1, and are suppressed by the HDJ-2 chaperone. Hum Mol Genet (1999) 1.98
Assembly of functional CFTR chloride channels. Annu Rev Physiol (2005) 1.95
Effects of heat shock, heat shock protein 40 (HDJ-2), and proteasome inhibition on protein aggregation in cellular models of Huntington's disease. Proc Natl Acad Sci U S A (2000) 1.88
Human macrophage scavenger receptors: primary structure, expression, and localization in atherosclerotic lesions. Proc Natl Acad Sci U S A (1990) 1.87
Mechanisms of arsenic-induced prolongation of cardiac repolarization. Mol Pharmacol (2004) 1.83
A foldable CFTR{Delta}F508 biogenic intermediate accumulates upon inhibition of the Hsc70-CHIP E3 ubiquitin ligase. J Cell Biol (2004) 1.70
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
BAG-2 acts as an inhibitor of the chaperone-associated ubiquitin ligase CHIP. Mol Biol Cell (2005) 1.52
The cochaperone HspBP1 inhibits the CHIP ubiquitin ligase and stimulates the maturation of the cystic fibrosis transmembrane conductance regulator. Mol Biol Cell (2004) 1.52
Regulation of the cytoplasmic quality control protein degradation pathway by BAG2. J Biol Chem (2005) 1.46
Acid denaturation and refolding of green fluorescent protein. Biochemistry (2004) 1.46
Novel mechanism associated with an inherited cardiac arrhythmia: defective protein trafficking by the mutant HERG (G601S) potassium channel. Circulation (1999) 1.42
HSP40 binding is the first step in the HSP90 chaperoning pathway for the progesterone receptor. J Biol Chem (2002) 1.41
Degradation of trafficking-defective long QT syndrome type II mutant channels by the ubiquitin-proteasome pathway. J Biol Chem (2005) 1.35
Chemical and biological folding contribute to temperature-sensitive DeltaF508 CFTR trafficking. Traffic (2008) 1.34
Human DnaJ homologs dj2 and dj3, and bag-1 are positive cochaperones of hsc70. J Biol Chem (2000) 1.34
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
The triage of damaged proteins: degradation by the ubiquitin-proteasome pathway or repair by molecular chaperones. FASEB J (2006) 1.23
Multiple 40-kDa heat-shock protein chaperones function in Tom70-dependent mitochondrial import. Mol Biol Cell (2007) 1.20
Pharmacological rescue of human K(+) channel long-QT2 mutations: human ether-a-go-go-related gene rescue without block. Circulation (2002) 1.18
Functional divergence between co-chaperones of Hsc70. J Biol Chem (2008) 1.17
Thapsigargin selectively rescues the trafficking defective LQT2 channels G601S and F805C. J Biol Chem (2003) 1.16
Retention in the endoplasmic reticulum as a mechanism of dominant-negative current suppression in human long QT syndrome. J Mol Cell Cardiol (2000) 1.13
Defining the requirements for Hsp40 and Hsp70 in the Hsp90 chaperone pathway. J Biol Chem (2006) 1.12
The Bcl-2 regulator FKBP38-calmodulin-Ca2+ is inhibited by Hsp90. J Biol Chem (2007) 1.00
Identification of a COOH-terminal segment involved in maturation and stability of human ether-a-go-go-related gene potassium channels. J Biol Chem (2003) 0.99
Peripheral protein quality control removes unfolded CFTR from the plasma membrane. Science (2010) 2.92
Regulation of Hsp70 function by HspBP1: structural analysis reveals an alternate mechanism for Hsp70 nucleotide exchange. Mol Cell (2005) 1.94
Post-translational integration of tail-anchored proteins is facilitated by defined molecular chaperones. J Cell Sci (2007) 1.53
Regulation of activation-induced deaminase stability and antibody gene diversification by Hsp90. J Exp Med (2010) 1.33
Mitochondrion-dependent N-terminal processing of outer membrane Mcl-1 protein removes an essential Mule/Lasu1 protein-binding site. J Biol Chem (2011) 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
Cofactor Tpr2 combines two TPR domains and a J domain to regulate the Hsp70/Hsp90 chaperone system. EMBO J (2003) 1.25
Heterogeneity of sodium current in atrial vs epicardial ventricular myocytes of adult guinea pig hearts. J Mol Cell Cardiol (2002) 1.25
Multiple 40-kDa heat-shock protein chaperones function in Tom70-dependent mitochondrial import. Mol Biol Cell (2007) 1.20
Hsp90 functions in the targeting and outer membrane translocation steps of Tom70-mediated mitochondrial import. J Biol Chem (2006) 1.20
Essential role of the unusual DNA-binding motif of BAG-1 for inhibition of the glucocorticoid receptor. J Biol Chem (2002) 1.19
Differential distribution of Kir2.1 and Kir2.3 subunits in canine atrium and ventricle. Am J Physiol Heart Circ Physiol (2002) 1.18
Functional divergence between co-chaperones of Hsc70. J Biol Chem (2008) 1.17
Spiral wave generation in heterogeneous excitable media. Phys Rev Lett (2002) 1.16
Prediction of novel Bag-1 homologs based on structure/function analysis identifies Snl1p as an Hsp70 co-chaperone in Saccharomyces cerevisiae. J Biol Chem (2002) 1.07
Global organization of dynamics in oscillatory heterogeneous excitable media. Phys Rev Lett (2005) 1.07
The heat shock protein 90-targeting drug cisplatin selectively inhibits steroid receptor activation. Mol Endocrinol (2003) 1.00
Identification of a COOH-terminal segment involved in maturation and stability of human ether-a-go-go-related gene potassium channels. J Biol Chem (2003) 0.99
Heat shock protein 70 inhibitors. 1. 2,5'-thiodipyrimidine and 5-(phenylthio)pyrimidine acrylamides as irreversible binders to an allosteric site on heat shock protein 70. J Med Chem (2014) 0.97
Inhibition of GR-mediated transcription by p23 requires interaction with Hsp90. FEBS Lett (2004) 0.97
Targeting and insertion of the cholesterol-binding translocator protein into the outer mitochondrial membrane. Biochemistry (2009) 0.96
Mitochondrial carrier protein biogenesis: role of the chaperones Hsc70 and Hsp90. Biochem J (2009) 0.95
Calcineurin B homologous protein 3 promotes the biosynthetic maturation, cell surface stability, and optimal transport of the Na+/H+ exchanger NHE1 isoform. J Biol Chem (2008) 0.95
Spontaneous initiation and termination of complex rhythms in cardiac cell culture. J Cardiovasc Electrophysiol (2003) 0.92
C-terminal domain of Kv4.2 and associated KChIP2 interactions regulate functional expression and gating of Kv4.2. J Biol Chem (2006) 0.89
The role of heterogeneities and intercellular coupling in wave propagation in cardiac tissue. Philos Trans A Math Phys Eng Sci (2006) 0.88
The DNAJA2 substrate release mechanism is essential for chaperone-mediated folding. J Biol Chem (2012) 0.87
Stochastic and spatial influences on drug-induced bifurcations in cardiac tissue culture. Phys Rev Lett (2009) 0.86
Function of cytosolic chaperones in Tom70-mediated mitochondrial import. Protein Pept Lett (2011) 0.86
OEP61 is a chaperone receptor at the plastid outer envelope. Biochem J (2011) 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
Compounds that correct F508del-CFTR trafficking can also correct other protein trafficking diseases: an in vitro study using cell lines. Orphanet J Rare Dis (2013) 0.85
Interaction between the human mitochondrial import receptors Tom20 and Tom70 in vitro suggests a chaperone displacement mechanism. J Biol Chem (2011) 0.84
Propagation through heterogeneous substrates in simple excitable media models. Chaos (2002) 0.84
Chaotic dynamics in cardiac aggregates induced by potassium channel block. Chaos (2012) 0.81
Reentrant waves in a ring of embryonic chick ventricular cells imaged with a Ca2+ sensitive dye. Biosystems (2003) 0.81
Chaperones and transcriptional regulation by nuclear receptors. Nat Struct Biol (2002) 0.80
Human mitochondrial import receptor Tom70 functions as a monomer. Biochem J (2010) 0.79
A G protein-coupled receptor and the intracellular synthase of its agonist functionally cooperate. J Cell Biol (2014) 0.79
New methods for the analysis of heartbeat behavior in risk stratification. Front Physiol (2011) 0.78
Aldosterone signaling through transient receptor potential melastatin 7 cation channel (TRPM7) and its α-kinase domain. Cell Signal (2013) 0.78
N-myristoylation and Ca2+ binding of calcineurin B homologous protein CHP3 are required to enhance Na+/H+ exchanger NHE1 half-life and activity at the plasma membrane. J Biol Chem (2012) 0.78
Pacemaker interactions induce reentrant wave dynamics in engineered cardiac culture. Chaos (2012) 0.77
Phase resetting and dynamics in isolated atrioventricular nodal cell clusters. Chaos (1995) 0.75
Scaling properties of conduction velocity in heterogeneous excitable media. Phys Rev E Stat Nonlin Soft Matter Phys (2011) 0.75
Functional characterization of oscillatory and excitable media. Bull Math Biol (2014) 0.75
Global organization of dynamics in cultured cardiac monolayers. Chaos (2004) 0.75