Published in Circulation on May 04, 1999
Long QT syndrome: from channels to cardiac arrhythmias. J Clin Invest (2005) 2.71
Drug-induced long QT syndrome. Pharmacol Rev (2010) 2.55
Impaired endocytosis of the ion channel TRPM4 is associated with human progressive familial heart block type I. J Clin Invest (2009) 2.54
The delicate balance between secreted protein folding and endoplasmic reticulum-associated degradation in human physiology. Physiol Rev (2012) 1.95
Trafficking-competent KCNQ1 variably influences the function of HERG long QT alleles. Heart Rhythm (2010) 1.61
Drug-induced QT interval shortening: potential harbinger of proarrhythmia and regulatory perspectives. Br J Pharmacol (2009) 1.57
A KCNQ1 mutation contributes to the concealed type 1 long QT phenotype by limiting the Kv7.1 channel conformational changes associated with protein kinase A phosphorylation. Heart Rhythm (2013) 1.46
Molecular and genetic basis of sudden cardiac death. J Clin Invest (2013) 1.23
Rescue of aberrant gating by a genetically encoded PAS (Per-Arnt-Sim) domain in several long QT syndrome mutant human ether-á-go-go-related gene potassium channels. J Biol Chem (2011) 1.10
Hsp40 chaperones promote degradation of the HERG potassium channel. J Biol Chem (2009) 1.09
Mechanisms of cardiac potassium channel trafficking. J Physiol (2007) 1.09
Compound heterozygous mutations P336L and I1660V in the human cardiac sodium channel associated with the Brugada syndrome. Circulation (2006) 1.08
Fluorescence-tracking of activation gating in human ERG channels reveals rapid S4 movement and slow pore opening. PLoS One (2010) 1.02
Food deprivation attenuates seizures through CaMKII and EAG K+ channels. PLoS Genet (2007) 0.95
Cardiac Arrhythmia: In vivo screening in the zebrafish to overcome complexity in drug discovery. Expert Opin Drug Discov (2010) 0.89
A Drosophila behavioral mutant, down and out (dao), is defective in an essential regulator of Erg potassium channels. Proc Natl Acad Sci U S A (2010) 0.87
Properties of WT and mutant hERG K(+) channels expressed in neonatal mouse cardiomyocytes. Am J Physiol Heart Circ Physiol (2010) 0.86
Reduced response to IKr blockade and altered hERG1a/1b stoichiometry in human heart failure. J Mol Cell Cardiol (2015) 0.86
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
Molecular Pathophysiology of Congenital Long QT Syndrome. Physiol Rev (2017) 0.85
Modulation of hERG channels by 5-(((1H-indazol-5-yl)oxy)methyl)-N-(4-(trifluoromethoxy)phenyl)pyrimidin-2-amine (ITP-2), a novel small molecule activator. Br J Pharmacol (2017) 0.84
Rescue of a trafficking defective human pacemaker channel via a novel mechanism: roles of Src, Fyn, and Yes tyrosine kinases. J Biol Chem (2009) 0.83
Effect of microculture on cell metabolism and biochemistry: do cells get stressed in microchannels? Anal Chem (2013) 0.81
Interaction between the cardiac rapidly (IKr) and slowly (IKs) activating delayed rectifier potassium channels revealed by low K+-induced hERG endocytic degradation. J Biol Chem (2011) 0.81
Emerging concepts in the pharmacogenomics of arrhythmias: ion channel trafficking. Expert Rev Cardiovasc Ther (2010) 0.78
Electrophysiological and fluorescence microscopy studies with HERG channel/EGFP fusion proteins. J Membr Biol (2008) 0.78
Cotranslational association of mRNA encoding subunits of heteromeric ion channels. Proc Natl Acad Sci U S A (2016) 0.77
Pharmacologic Approach to Defective Protein Trafficking in the E637K-hERG Mutant with PD-118057 and Thapsigargin. PLoS One (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
Cytoskeleton regulation of ion channels. Circulation (2015) 0.75
Association of the hERG mutation with long‑QT syndrome type 2, syncope and epilepsy. Mol Med Rep (2016) 0.75
Molecular pathogenesis of long QT syndrome type 2. J Arrhythm (2016) 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
Paul F. Cranefield award to Matthew Trudeau. J Gen Physiol (2014) 0.75
HERG, a human inward rectifier in the voltage-gated potassium channel family. Science (1995) 7.26
The DNA replication and damage checkpoint pathways induce transcription by inhibition of the Crt1 repressor. Cell (1998) 5.37
The SAD1/RAD53 protein kinase controls multiple checkpoints and DNA damage-induced transcription in yeast. Genes Dev (1994) 5.20
A component of calcium-activated potassium channels encoded by the Drosophila slo locus. Science (1991) 4.94
DNA polymerase epsilon links the DNA replication machinery to the S phase checkpoint. Cell (1995) 4.90
Diagnostic risk adjustment for Medicaid: the disability payment system. Health Care Financ Rev (1996) 4.34
A controlled study of adenoviral-vector-mediated gene transfer in the nasal epithelium of patients with cystic fibrosis. N Engl J Med (1995) 4.30
Pre-mRNA splicing and mRNA export linked by direct interactions between UAP56 and Aly. Nature (2001) 3.94
Properties of HERG channels stably expressed in HEK 293 cells studied at physiological temperature. Biophys J (1998) 3.83
CD34+ hematopoietic progenitors from human cord blood differentiate along two independent dendritic cell pathways in response to GM-CSF+TNF alpha. J Exp Med (1996) 3.82
Translocation (8;21) in oligoblastic leukemia: is this a true myelodysplastic syndrome? Leuk Res (1994) 3.66
A primitive enantiornithine bird and the origin of feathers. Science (2000) 2.96
DNA damage and cell cycle regulation of ribonucleotide reductase. Bioessays (1993) 2.81
Fractional contribution of calcium to the cation current through glutamate receptor channels. Neuron (1993) 2.69
Risk of developing colorectal cancer and benign colorectal neoplasm in patients with chronic constipation. Aliment Pharmacol Ther (2014) 2.67
Fractional calcium currents through recombinant GluR channels of the NMDA, AMPA and kainate receptor subtypes. J Physiol (1995) 2.67
The prevalence of nocturia and its effect on health-related quality of life and sleep in a community sample in the USA. BJU Int (2003) 2.59
HERG channel dysfunction in human long QT syndrome. Intracellular transport and functional defects. J Biol Chem (1998) 2.52
Mutations in the human orthologue of the mouse underwhite gene (uw) underlie a new form of oculocutaneous albinism, OCA4. Am J Hum Genet (2001) 2.43
Inducible T cell antigen 4-1BB. Analysis of expression and function. J Immunol (1993) 2.40
Differential effect of steady versus oscillating flow on bone cells. J Biomech (1998) 2.38
Myosin heavy chain messenger RNA and protein isoform transitions during cardiac hypertrophy. Interaction between hemodynamic and thyroid hormone-induced signals. J Clin Invest (1987) 2.37
Adverse effects of low dose amiodarone: a meta-analysis. J Am Coll Cardiol (1997) 2.37
Cystic fibrosis gene mutations and pancreatitis risk: relation to epithelial ion transport and trypsin inhibitor gene mutations. Gastroenterology (2001) 2.36
Safety and biological efficacy of a lipid-CFTR complex for gene transfer in the nasal epithelium of adult patients with cystic fibrosis. Mol Ther (2000) 2.34
MAP kinase-related FUS3 from S. cerevisiae is activated by STE7 in vitro. Nature (1993) 2.33
Oxygen-bridged dinuclear ruthenium amine complex specifically inhibits Ca2+ uptake into mitochondria in vitro and in situ in single cardiac myocytes. J Biol Chem (1998) 2.32
Correction of defective protein trafficking of a mutant HERG potassium channel in human long QT syndrome. Pharmacological and temperature effects. J Biol Chem (1999) 2.27
Conotruncal anomaly face syndrome is associated with a deletion within chromosome 22q11. J Med Genet (1993) 2.27
Functional association of U2 snRNP with the ATP-independent spliceosomal complex E. Mol Cell (2000) 2.27
The smallest known non-avian theropod dinosaur. Nature (2000) 2.26
A novel mutation in the cystic fibrosis gene in patients with pulmonary disease but normal sweat chloride concentrations. N Engl J Med (1994) 2.25
Lessons from loricrin-deficient mice: compensatory mechanisms maintaining skin barrier function in the absence of a major cornified envelope protein. J Cell Biol (2000) 2.22
Function of the Escherichia coli msbB gene, a multicopy suppressor of htrB knockouts, in the acylation of lipid A. Acylation by MsbB follows laurate incorporation by HtrB. J Biol Chem (1997) 2.17
Mice devoid of gamma-aminobutyrate type A receptor beta3 subunit have epilepsy, cleft palate, and hypersensitive behavior. Proc Natl Acad Sci U S A (1997) 2.16
Investigation of the mechanism of alcohol-induced bronchial asthma. J Allergy Clin Immunol (1996) 2.16
Benefit of bilateral over single internal mammary artery grafts for multiple coronary artery bypass grafting. Circulation (2001) 2.15
PEGylated PLGA nanoparticles as protein carriers: synthesis, preparation and biodistribution in rats. J Control Release (2001) 2.15
Clinical outcome after endoscopic mucosal resection for esophageal squamous cell carcinoma invading the muscularis mucosae--a multicenter retrospective cohort study. Endoscopy (2007) 2.11
Mechanism of block and identification of the verapamil binding domain to HERG potassium channels. Circ Res (1999) 2.10
Ribonucleotide reductase: regulation, regulation, regulation. Trends Biochem Sci (1992) 2.07
Two isoforms of the mouse ether-a-go-go-related gene coassemble to form channels with properties similar to the rapidly activating component of the cardiac delayed rectifier K+ current. Circ Res (1997) 1.98
High frequency class switching of an IgM+ B lymphoma clone CH12F3 to IgA+ cells. Int Immunol (1996) 1.97
Regulation of deactivation by an amino terminal domain in human ether-à-go-go-related gene potassium channels. J Gen Physiol (1998) 1.95
Odontogenic tumors. A demographic study of 759 cases in a Chinese population. Oral Surg Oral Med Oral Pathol Oral Radiol Endod (1998) 1.93
The impact on health-related quality of life of stress, urge and mixed urinary incontinence. BJU Int (2003) 1.93
Coronary arteriovenous fistula. Nine operated cases. Am Heart J (1966) 1.89
Occupational exposure to bisphenol-A (BPA) and the risk of self-reported male sexual dysfunction. Hum Reprod (2009) 1.87
A precision measurement of the mass of the top quark. Nature (2004) 1.87
Making risk adjustment work for everyone. Inquiry (1995) 1.85
VI. Genome structure and cognitive map of Williams syndrome. J Cogn Neurosci (2000) 1.83
Fractalkine and macrophage-derived chemokine: T cell-attracting chemokines expressed in T cell area dendritic cells. Eur J Immunol (1999) 1.82
Pulmonary epithelial sodium-channel dysfunction and excess airway liquid in pseudohypoaldosteronism. N Engl J Med (1999) 1.81
The Community Medical Alliance: an integrated system of care in Greater Boston for people with severe disability and AIDS. Manag Care Q (1996) 1.81
Proteolytic processing mechanisms in the biosynthesis of neuroendocrine peptides: the subtilisin-like proprotein convertases. Front Neuroendocrinol (1995) 1.79
Rapid disease progression correlates with instability of mutant SOD1 in familial ALS. Neurology (2005) 1.78
Translocation of C. elegans CED-4 to nuclear membranes during programmed cell death. Science (2000) 1.77
A case of Rothmund-Thomson syndrome with reduced DNA repair capacity. Arch Dermatol (1993) 1.76
Requirement for B cell linker protein (BLNK) in B cell development. Science (1999) 1.74
Temperature dependence of early and late currents in human cardiac wild-type and long Q-T DeltaKPQ Na+ channels. Am J Physiol (1998) 1.74
Aortic valve prolapse and aortic regurgitation associated with subpulmonic ventricular septal defect. Am J Cardiol (1997) 1.74
Breast cancer metastatic potential correlates with a breakdown in homospecific and heterospecific gap junctional intercellular communication. Cancer Res (2001) 1.72
Transfer of rapid inactivation and sensitivity to the class III antiarrhythmic drug E-4031 from HERG to M-eag channels. J Physiol (1998) 1.72
Covalent regulation of ULVWF string formation and elongation on endothelial cells under flow conditions. J Thromb Haemost (2008) 1.68
Developmentally regulated expression of the PD-1 protein on the surface of double-negative (CD4-CD8-) thymocytes. Int Immunol (1996) 1.68
Drug-induced long QT syndrome: hERG K+ channel block and disruption of protein trafficking by fluoxetine and norfluoxetine. Br J Pharmacol (2006) 1.68
Background current in sino-atrial node cells of the rabbit heart. J Physiol (1992) 1.66
Angiotensin II type 2 receptor mediates vascular smooth muscle cell apoptosis in cystic medial degeneration associated with Marfan's syndrome. Circulation (2001) 1.63
Mechanically stimulated osteocytes regulate osteoblastic activity via gap junctions. Am J Physiol Cell Physiol (2006) 1.63
Differential involvement of the hyaluronan (HA) receptors CD44 and receptor for HA-mediated motility in endothelial cell function and angiogenesis. J Biol Chem (2001) 1.60
A new method for the quantitative standardization of cross-sectional areas of the pulmonary arteries in congenital heart diseases with decreased pulmonary blood flow. J Thorac Cardiovasc Surg (1984) 1.60
Disulfide bond reduction of von Willebrand factor by ADAMTS-13. J Thromb Haemost (2010) 1.59
The human OX40/gp34 system directly mediates adhesion of activated T cells to vascular endothelial cells. J Exp Med (1996) 1.59
4-1BB T-cell antigen binds to mature B cells and macrophages, and costimulates anti-mu-primed splenic B cells. Eur J Immunol (1994) 1.58
Interruption of the aortic arch associated with deletion of chromosome 22q11 is associated with a subarterial and doubly committed ventricular septal defect in Japanese patients. Cardiol Young (1999) 1.58
Spontaneous autoimmune skin lesions of MRL/n mice: autoimmune disease-prone genetic background in relation to Fas-defect MRL/1pr mice. J Invest Dermatol (1996) 1.57
Are nonfunctioning pituitary adenomas extending into the cavernous sinus aggressive and/or invasive? Neurosurgery (2001) 1.57
Effect of sulphasalazine and its metabolites on the generation of reactive oxygen species. Gut (1987) 1.57
Correlation between operative outcomes of cervical compression myelopathy and mri of the spinal cord. Spine (Phila Pa 1976) (2001) 1.57
Age-dependent prevalence of mutations at the GLC1A locus in primary open-angle glaucoma. Am J Ophthalmol (2000) 1.57
Ca(2+)-independent but voltage-dependent secretion in mammalian dorsal root ganglion neurons. Nat Neurosci (2002) 1.56
Modulation of norepinephrine release by ATP-dependent K(+)-channel activators and inhibitors in guinea-pig and human isolated right atrium. Cardiovasc Res (1999) 1.56
Functional gap junctions between osteocytic and osteoblastic cells. J Bone Miner Res (2000) 1.55
Purification and characterization of choline oxidase from Arthrobacter globiformis. J Biochem (1977) 1.55
Systemically administered interleukin-10 reduces tumor necrosis factor-alpha production and significantly improves functional recovery following traumatic spinal cord injury in rats. J Neurotrauma (1999) 1.55
ADP-ribosylation of the rhoA gene product by botulinum C3 exoenzyme causes Swiss 3T3 cells to accumulate in the G1 phase of the cell cycle. Oncogene (1993) 1.55
Health-related quality of life among adults with symptoms of overactive bladder: results from a U.S. community-based survey. Urology (2001) 1.53
Germline mutations in an intermediate chain dynein cause primary ciliary dyskinesia. Am J Respir Cell Mol Biol (2001) 1.50
Cardiac-specific overexpression of cyclin-dependent kinase 2 increases smaller mononuclear cardiomyocytes. Circ Res (2001) 1.50
Human eHAND, but not dHAND, is down-regulated in cardiomyopathies. J Mol Cell Cardiol (2001) 1.50