Long QT syndrome 2

Common Name(s)

Long QT syndrome 2

Congenital long QT syndrome is electrocardiographically characterized by a prolonged QT interval and polymorphic ventricular arrhythmias (torsade de pointes). These cardiac arrhythmias may result in recurrent syncope, seizure, or sudden death ({7:Jongbloed et al., 1999}).
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Long QT syndrome 2" for support, advocacy or research.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Long QT syndrome 2" returned 33 free, full-text research articles on human participants. First 3 results:

Large-scale mutational analysis of Kv11.1 reveals molecular insights into type 2 long QT syndrome.
 

Author(s): Corey L Anderson, Catherine E Kuzmicki, Ryan R Childs, Caleb J Hintz, Brian P Delisle, Craig T January

Journal:

 

It has been suggested that deficient protein trafficking to the cell membrane is the dominant mechanism associated with type 2 Long QT syndrome (LQT2) caused by Kv11.1 potassium channel missense mutations, and that for many mutations the trafficking defect can be corrected pharmacologically. ...

Last Updated: 24 Nov 2014

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Re-trafficking of hERG reverses long QT syndrome 2 phenotype in human iPS-derived cardiomyocytes.
 

Author(s): Ashish Mehta, Glen Lester Sequiera, Chrishan J A Ramachandra, Yuliansa Sudibyo, Yingying Chung, Jingwei Sheng, Keng Yean Wong, Teng Hong Tan, Philip Wong, Reginald Liew, Winston Shim

Journal: Cardiovasc. Res.. 2014 Jun;102(3):497-506.

 

Long QT syndrome 2 (LQTS2) caused by missense mutations in hERG channel is clinically associated with abnormally prolonged ventricular repolarization and sudden cardiac deaths. Modelling monogenic arrhythmogenic diseases using human-induced pluripotent stem cells (hiPSCs) offers unprecedented ...

Last Updated: 23 May 2014

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Mechanistic basis for type 2 long QT syndrome caused by KCNH2 mutations that disrupt conserved arginine residues in the voltage sensor.
 

Author(s): Christie M McBride, Ashley M Smith, Jennifer L Smith, Allison R Reloj, Ellyn J Velasco, Jonathan Powell, Claude S Elayi, Daniel C Bartos, Don E Burgess, Brian P Delisle

Journal: J. Membr. Biol.. 2013 May;246(5):355-64.

 

KCNH2 encodes the Kv11.1 channel, which conducts the rapidly activating delayed rectifier K+ current (I Kr) in the heart. KCNH2 mutations cause type 2 long QT syndrome (LQT2), which increases the risk for life-threatening ventricular arrhythmias. LQT2 mutations are predicted to prolong ...

Last Updated: 15 May 2013

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Long QT syndrome 2" returned 0 free, full-text review articles on human participants.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

Effect of GS-6615 on QT, Safety, and Tolerability in Adults With Long QT2 Syndrome
 

Status: Recruiting

Condition Summary: LQT2 Syndrome

 

Last Updated: 11 Aug 2015

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Comparison Between Epinephrine and Exercise Test in QT Long Syndrome Patients
 

Status: Recruiting

Condition Summary: Long QT Syndrome Type 1 or 2

 

Last Updated: 23 May 2014

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