Congenital LQT syndromes: From gene to Torsade de Pointes

Korean Journal of Physiology and Pharmacology

Volumn 6, Issue 1, 2002, Pages 1-7

  Carmeliet, Edward ^a  

^a UNIVERSITY OF LEUVEN   (Belgium)

Author keywords

block; Arrhythmia; Congenital LQT; Delayed K+ currents; Persistent Na+ current; Torsade de Pointes

Indexed keywords

1 [2 (6 METHYL 2 PYRIDYL)ETHYL] 4 (4 METHYLSULFONYLAMINOBENZOYL)PIPERIDINE; ADRENALIN; ALDOSTERONE; BETA ADRENERGIC RECEPTOR BLOCKING AGENT; DOFETILIDE; FLECAINIDE; ISOPRENALINE; LIDOCAINE; PILSICAINIDE; POTASSIUM ION; RENIN; SODIUM CHANNEL; SODIUM CHANNEL BLOCKING AGENT; SODIUM ION;

ANIMAL CELL; ANIMAL EXPERIMENT; AROUSAL; ARRHYTHMOGENESIS; ARTICLE; ARTIFICIAL HEART PACEMAKER; CONTROLLED STUDY; DEFIBRILLATOR; ELECTROCARDIOGRAM; EMOTION; EXERCISE; GENE MUTATION; HEART DEPOLARIZATION; HEART MUSCLE POTENTIAL; HEART REPOLARIZATION; HEART VENTRICLE WALL; HUMAN; LONG QT SYNDROME; NONHUMAN; PATHOGENESIS; POTASSIUM CURRENT; REENTRY ARRHYTHMIA; REST; SLEEP; SUDDEN DEATH; SWIMMING; TORSADE DES POINTES;

EID: 0036127953     PISSN: 12264512     EISSN: None     Source Type: Journal    
DOI: None     Document Type: Article

References (52)

1. MiRP1 forms IKr potassium channels with HERG and is associated with cardiac arrhythmia
2. + channel
3. Clinical and genetic variables associated with acute arousal and nonarousal-related cardiac events among subjects with long QT syndrome
4. 1-subunits
5. Altered potassium balance and aldosterone secretion in a mouse model of human congenital long QT syndrome
6. The cardiac sodium channel: Gating function and molecular pharmacology
7. Differential response to right and left ansae subclaviae stimulation of early afterdepolarizations and ventricular tachycardia induced by cesium in dogs
8. Effects of flecainide in patients with new SCN5A mutation: Mutation-specific therapy for long-QT syndrome?
9. Ks suppression in LQT1 mutants
10. Right bundle branch block, persistent ST segment elevation and sudden cardiac death: A distinct clinical and electrocardiographic syndrome. A multicenter report
11. Ks does not induce early afterdepolarization activity but promotes beta-adrenergic agonist-induced delayed afterdepolarization activity
12. Targeted disruption of the Kcnq1 gene produces a mouse model of Jervell and Lange-Nielsen Syndrome
13. + channels expressed in mammalian cells
14. + channel mutations in Romano-Ward and Jervell and Lange-Nielsen inherited cardiac arrhythmias
15. Effect of tetrodotoxin on action potentials of the conducting system in the dog heart
16. La tachycardie ventriculaire à deux foyers opposés variables
17. Electrophysiological mechanism of the characteristic electrocardiographic morphology of torsade de pointes tachyarrhythmias in the long-QT syndrome: Detailed analysis of ventricular tridimensional activation patterns
18. Retention in the endoplasmic reticulum as a mechanism of dominant-negative current suppression in human long QT syndrome
19. Novel characteristics of a misprocessed mutant HERG channel linked to hereditary long QT syndrome
20. Effects of unilateral stellate ganglion block on the spectral characteristics of heart rate variability
21. A spectrum of functional effects for disease causing mutations in the Jervell and Lange-Nielsen syndrome
22. The dominant negative LQT2 mutation A561V reduces wild-type HERG expression
23. A revised view of cardiac sodium channel "blockade" in the long-QT syndrome
24. The roles of ion channels in an inherited heart disease: Molecular genetics of the long QT syndrome
25. + channel-related long-QT syndrome: Altered gating and selectivity in the HERG1 N629D mutant
26. Effectiveness and limitations of β-blocker therapy in congenital long-QT syndrome
27. c antiarrhythmic flecainide
28. Novel mechanism of HERG current suppression in LQT2: Shift in voltage dependence of HERG inactivation
29. Characterization of S818L mutation in HERG C-terminus in LQT2. Modification of activation-deactivation gating properties
30. Differential response of dynamic ventricular repolarization to sympathetic stimulation in LQT1, LQT2 and LQT3 forms of congenital Long QT syndrome
31. Abrupt rate accelerations or premature beats cause life-threatening arrhythmias in mice with long-QT3 syndrome
32. + channel mutant by pilsicainide and lidocaine with distinct mechanisms
33. + channel blockade, β-adrenergic stimulation, and rapid pacing in a cellular model mimicking the SCN5A and HERG defects present in the long-QT syndrome
34. Recent advances in understanding the molecular mechanisms of the long QT syndrome
35. + channel blockade and to increases in heart rate. Implications for gene-specific therapy
36. Genotype-phenotype correlation in the long-QT syndrome: Gene-specific triggers for life-threatening arrhythmias
37. Identification of specific pore residues mediating KCNQ1 inactivation. A novel mechanism for long QT syndrome
38. Ks channels and channels formed with two minK mutants that cause long QT syndrome
39. Cellular basis for the ECG features of the LQT1 form of the long-QT syndrome. Effects of β-adrenergic agonists and antagonists and sodium channel blockers on transmural dispersion of repolarization and torsade de pointes
40. Cellular basis for long QT, transmural dispersion of repolarization, and torsade de pointes in the long QT syndrome
41. Differential effects of β-adrenergic agonists and antagonists in LQT1, LQT2 and LQT3 models of the long QT syndrome
42. Gene-specific differences in the circadian variation of ventricular repolarization in the long QT syndrome: A key to sudden death during sleep?
43. + channel
44. Inner ear defects induced by null mutation of the risk gene
45. Cardiac pacing in the long QT syndrome: Review of available data and practical recommendations
46. Gating-dependent mechanisms for flecainide action in SCN5A-linked arrhythmia syndromes
47. Pause induced early afterdepolarizations in the long QT syndrome: A simulation study
48. + channel α-subunit: A computational analysis
49. Heterozygous SCN5A mutation in the domain I-II linker causes long QT syndrome with 2 : 1 atrioventricular block
50. 2+ current: A cellular mechanism for long Q-T arrhythmias
51. Calmodulin kinase inhibition prevents development of the arrhythmogenic transient inward current
52. HERG channel dysfunction in human long QT syndrome. Intracellular transport and functional defects