scholarly journals A novel transgenic rabbit model with reduced repolarization reserve: long QT syndrome caused by a dominant-negative mutation of theKCNE1gene

2016 ◽  
Vol 173 (12) ◽  
pp. 2046-2061 ◽  
Author(s):  
Péter Major ◽  
István Baczkó ◽  
László Hiripi ◽  
Katja E. Odening ◽  
Viktor Juhász ◽  
...  
Keyword(s):  
Long Qt Syndrome ◽  
Rabbit Model ◽  
Dominant Negative ◽  
Long Qt ◽  
Transgenic Rabbit ◽  
Dominant Negative Mutation ◽  
Repolarization Reserve ◽  
Qt Syndrome

scholarly journals Nicorandil normalizes prolonged repolarisation in the first transgenic rabbit model with Long-QT syndrome 1 both in vitro and in vivo

2011 ◽  
Vol 650 (1) ◽  
pp. 309-316 ◽  
Author(s):  
Jürgen Biermann ◽  
Kezhong Wu ◽  
Katja E. Odening ◽  
Stefan Asbach ◽  
Gideon Koren ◽  
...  
Keyword(s):  
Long Qt Syndrome ◽  
Rabbit Model ◽  
Long Qt ◽  
Transgenic Rabbit ◽  
Qt Syndrome

scholarly journals Origin of complex behaviour of spatially discordant alternans in a transgenic rabbit model of type 2 long QT syndrome

2009 ◽  
Vol 587 (19) ◽  
pp. 4661-4680 ◽  
Author(s):  
Ohad Ziv ◽  
Eduardo Morales ◽  
Yoon-kyu Song ◽  
Xuwen Peng ◽  
Katja E. Odening ◽  
...  
Keyword(s):  
Long Qt Syndrome ◽  
Rabbit Model ◽  
Long Qt ◽  
Complex Behaviour ◽  
Transgenic Rabbit ◽  
Qt Syndrome

Neonatal Long QT Syndrome Due to a De Novo Dominant Negative hERG Mutation

2007 ◽  
Vol 16 (4) ◽  
pp. 416-412 ◽  
Author(s):  
Theresa A. Beery ◽  
Kerry A. Shooner ◽  
D. Woodrow Benson
Keyword(s):  
Long Qt Syndrome ◽  
De Novo ◽  
Sinus Bradycardia ◽  
Molecular Genetic ◽  
Ventricular Tachyarrhythmia ◽  
Dominant Negative ◽  
De Novo Mutation ◽  
Long Qt ◽  
Dominant Negative Mutation ◽  
Qt Syndrome

A 4-day-old girl with ventricular tachyarrhythmias, sinus bradycardia, and 2:1 atrioventricular block had prolongation of the QT interval. She was symptomatic with arching, gasping, and cyanosis presumably due to a life-threatening ventricular tachyarrhythmia such as torsades de pointes. Molecular genetic studies indicated a heterozygous, de novo, dominant negative mutation in hERG, a gene that encodes a protein in a potassium ion channel. The parents do not have the mutation. The patient’s clinical scenario was produced by the convergence of 3 events: a de novo mutation occurred in hERG, the mutation was dominant negative, and the action of the mutation resulted in neonatal long QT syndrome. The child was treated aggressively and is doing well at age 6 years.

scholarly journals Complex excitation dynamics underlie polymorphic ventricular tachycardia in a transgenic rabbit model of long QT syndrome type 1

Heart Rhythm ◽  
2015 ◽  
Vol 12 (1) ◽  
pp. 220-228 ◽  
Author(s):  
Tae Yun Kim ◽  
Yukiko Kunitomo ◽  
Zachary Pfeiffer ◽  
Divyang Patel ◽  
Jungmin Hwang ◽  
...  
Keyword(s):  
Ventricular Tachycardia ◽  
Long Qt Syndrome ◽  
Rabbit Model ◽  
Polymorphic Ventricular Tachycardia ◽  
Syndrome Type ◽  
Long Qt ◽  
Transgenic Rabbit ◽  
Qt Syndrome ◽  
Excitation Dynamics

scholarly journals Late I Na Blocker GS967 Supresses Polymorphic Ventricular Tachycardia in a Transgenic Rabbit Model of Long QT Type 2

2020 ◽  
Vol 13 (8) ◽  
Author(s):  
Jungmin Hwang ◽  
Tae Yun Kim ◽  
Dmitry Terentyev ◽  
Mingwang Zhong ◽  
Anatoli Y. Kabakov ◽  
...  
Keyword(s):  
Action Potential ◽  
Long Qt Syndrome ◽  
Rabbit Model ◽  
Syndrome Type ◽  
Long Qt ◽  
Repolarization Reserve ◽  
Action Potential Plateau ◽  
Qt Syndrome ◽  
Potential Plateau

Background: Long QT syndrome has been associated with sudden cardiac death likely caused by early afterdepolarizations (EADs) and polymorphic ventricular tachycardias (PVTs). Suppressing the late sodium current (I NaL ) may counterbalance the reduced repolarization reserve in long QT syndrome and prevent EADs and PVTs. Methods: We tested the effects of the selective I NaL blocker GS967 on PVT induction in a transgenic rabbit model of long QT syndrome type 2 using intact heart optical mapping, cellular electrophysiology and confocal Ca 2+ imaging, and computer modeling. Results: GS967 reduced ventricular fibrillation induction under a rapid pacing protocol (n=7/14 hearts in control versus 1/14 hearts at 100 nmol/L) without altering action potential duration or restitution and dispersion. GS967 suppressed PVT incidences by reducing Ca 2+ -mediated EADs and focal activity during isoproterenol perfusion (at 30 nmol/L, n=7/12 and 100 nmol/L n=8/12 hearts without EADs and PVTs). Confocal Ca 2+ imaging of long QT syndrome type 2 myocytes revealed that GS967 shortened Ca 2+ transient duration via accelerating Na + /Ca 2+ exchanger (I NCX )-mediated Ca 2+ efflux from cytosol, thereby reducing EADs. Computer modeling revealed that I NaL potentiates EADs in the long QT syndrome type 2 setting through (1) providing additional depolarizing currents during action potential plateau phase, (2) increasing intracellular Na + (Na i ) that decreases the depolarizing I NCX thereby suppressing the action potential plateau and delaying the activation of slowly activating delayed rectifier K + channels (I Ks ), suggesting important roles of I NaL in regulating Na i . Conclusions: Selective I NaL blockade by GS967 prevents EADs and abolishes PVT in long QT syndrome type 2 rabbits by counterbalancing the reduced repolarization reserve and normalizing Na i . Graphic Abstract: A graphic abstract is available for this article.

scholarly journals Alternating Cycle Lengths Increases Dispersion of Action Potential Durations (APD) in Transgenic Rabbit Model of Long QT Syndrome Type 2

2012 ◽  
Vol 102 (3) ◽  
pp. 539a-540a
Author(s):  
Tae Yun Kim ◽  
Paul Jeng ◽  
Chantel Taylor ◽  
JungMin Hwang ◽  
Xuwen Peng ◽  
...  
Keyword(s):  
Action Potential ◽  
Long Qt Syndrome ◽  
Rabbit Model ◽  
Syndrome Type ◽  
Long Qt ◽  
Transgenic Rabbit ◽  
Cycle Lengths ◽  
Qt Syndrome

scholarly journals A Weak Dominant Negative Mutation of KCNQ1-G269S Affects PKA-Mediated Up-Regulation of IKs Channels and Causes Adrenergic Triggered Long QT Syndrome

2011 ◽  
Vol 27 (Supplement) ◽  
pp. OP60_3
Author(s):  
Jie Wu ◽  
Nobu Naiki ◽  
Wei-Guang Ding ◽  
Hiroshi Matsuura ◽  
Minoru Horie
Keyword(s):  
Long Qt Syndrome ◽  
Dominant Negative ◽  
Long Qt ◽  
Dominant Negative Mutation ◽  
Qt Syndrome

scholarly journals Different Ventricular Fibrillation (VF) Dynamics in Long QT Syndrome Type 1 vs. 2 in a Transgenic Rabbit Model

2013 ◽  
Vol 104 (2) ◽  
pp. 294a-295a
Author(s):  
Tae Yun Kim ◽  
Jung Min Hwang ◽  
Zachary Pfeiffer ◽  
Yukiko Kunitomo ◽  
Xuwen Peng ◽  
...  
Keyword(s):  
Ventricular Fibrillation ◽  
Long Qt Syndrome ◽  
Rabbit Model ◽  
Syndrome Type ◽  
Long Qt ◽  
Transgenic Rabbit ◽  
Qt Syndrome

Long QT syndrome: reduced repolarization reserve and the genetic link

2006 ◽  
Vol 259 (1) ◽  
pp. 59-69 ◽  
Author(s):  
D. M. RODEN
Keyword(s):  
Long Qt Syndrome ◽  
Long Qt ◽  
Genetic Link ◽  
Repolarization Reserve ◽  
Qt Syndrome

scholarly journals Takotsubo cardiomyopathy and the long-QT syndrome: an insult to repolarization reserve

EP Europace ◽  
2009 ◽  
Vol 11 (6) ◽  
pp. 697-700 ◽  
Author(s):  
E. R. Behr ◽  
S. Mahida
Keyword(s):  
Takotsubo Cardiomyopathy ◽  
Long Qt Syndrome ◽  
Long Qt ◽  
Repolarization Reserve ◽  
Qt Syndrome
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