Long QT syndrome 1

Common Name(s)

Long QT syndrome 1

Romano-Ward syndrome is the most common form of inherited long QT syndrome. Symptoms include arrhythmia, fainting, cardiac arrest, and sudden death. There are six different types of this syndrome, long QT 1 through 6. Each type is caused by a change in a different gene. The most prevalent form of long QT syndrome is long QT type 1. Long QT type 1 is caused by changes in the KCNQ1 gene. Romano-Ward syndrome is inherited in an autosomal dominant fashion.
 

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Condition Specific Organizations

Following organizations serve the condition "Long QT syndrome 1" for support, advocacy or research.

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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 1" returned 31 free, full-text research articles on human participants. First 3 results:

The role of CAV3 in long-QT syndrome: clinical and functional assessment of a caveolin-3/Kv11.1 double heterozygote versus caveolin-3 single heterozygote.
 

Author(s): Paula L Hedley, Jørgen K Kanters, Maja Dembic, Thomas Jespersen, Lasse Skibsbye, Frederik H Aidt, Ole Eschen, Claus Graff, Elijah R Behr, Sarah Schlamowitz, Valerie Corfield, William J McKenna, Michael Christiansen

Journal: Circ Cardiovasc Genet. 2013 Oct;6(5):452-61.

 

Mutations in CAV3, coding for caveolin-3, the major constituent scaffolding protein of cardiac caveolae, have been associated with skeletal muscle disease, cardiomyopathy, and most recently long-QT syndrome (LQTS) and sudden infant death syndrome. We examined the occurrence of CAV3 ...

Last Updated: 16 Oct 2013

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The interaction of caveolin 3 protein with the potassium inward rectifier channel Kir2.1: physiology and pathology related to long qt syndrome 9 (LQT9).
 

Author(s): Ravi Vaidyanathan, Amanda L Vega, Chunhua Song, Qing Zhou, Bi-Hua Tan, Bihua Tan, Stuart Berger, Jonathan C Makielski, Lee L Eckhardt

Journal: J. Biol. Chem.. 2013 Jun;288(24):17472-80.

 

Mutations in CAV3 cause LQT syndrome 9 (LQT9). A previously reported LQT9 patient had prominent U waves on ECG, a feature that has been correlated with Kir2.1 loss of function. Our objective was to determine whether caveolin 3 (Cav3) associates with Kir2.1 and whether LQT9-associated ...

Last Updated: 17 Jun 2013

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Clinical utility gene card for: long-QT syndrome (types 1-13).
 

Author(s): Britt-Maria Beckmann, Arthur A M Wilde, Stefan Kääb

Journal: Eur. J. Hum. Genet.. 2013 Oct;21(10):.

 

Last Updated: 19 Sep 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 1" returned 0 free, full-text review articles on human participants.

 
 
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Clinical Trial Information This information is provided by ClinicalTrials.gov

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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