What is Long QT Syndrome (LQTS)?LQTS occurs due to a disruption in the heart's ion channels regulating the electrical system in the heart. Symptoms of LQTS are fainting, seizures and cardiac arrest.
It is important to note that as many as 50% of patients are asymptomatic and often the first symptom is sudden cardiac death.
LQTS can be genetic, or acquired, and is a leading cause of sudden cardiac death in people under age forty.
Researchers have identified three major LQTS genes and 10 minor LQTS-susceptibility genes which account for nearly 80% of the affected population1, but its genetic component is still not completely understood. While my family has had genetic testing done, and we are able to trace who is at risk based on family histories of sudden cardiac death, the gene associated with our LQTS has not yet been identified.
Broader Impacts of LQTSBeyond its human cost, LQTS may be affecting the development of new drugs; "attenuation of the QT interval is one of most common reasons of US Food and Drug Administration black box warnings or medications being halted in development because of the risk of sudden cardiac death2." Without better understanding of the mechanisms behind LQTS, it is impossible to completely assess its impact on the drug discovery and development pipeline.
|Taconic Biosciences' Models of Interest:|
|Kcnj1 - Model 12079 - KO||Kcnj14||Kcna7|
|Kcnj1 - Model 11481 - cKO||Kcnj15||Kcna10|