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Dis Model Mech. 2011 January; 4(1): 2.
PMCID: PMC3014334

In vivo screen for suppressors of long QT syndrome

figure RH1
Figure reproduced from: Arnaout, R., Ferrer, T., Huisken, J., Spitzer, K., Stainier, D. Y., Tristani-Firouzi, M. and Chi, N. C. (2007). Zebrafish model for human long QT syndrome. Proc. Natl. Acad. Sci. USA 104, 11316–11321.

Long QT syndrome (LQTS) involves delayed repolarisation of the heart, causing heart problems or sudden death. It can be congenital (usually due to mutations in genes encoding cardiac ion channels, such as KCNH2) or acquired, and is also a side effect of many drugs, but there are limited treatment options. Among model organisms, the zebrafish offers many advantages for analysing LQTS. Peal et al. carried out an in vivo functional screen using the zebrafish breakdance mutant, which displays many features of human LQTS owing to a mutation in kcnh2, and identified two drugs that correct the cardiac phenotype. These results and future in vivo screens will contribute to the development of novel targeted therapeutics for LQTS patients.

Peal D.S., Mills R.W., Lynch S.N., Mosley J.M., Lim E., Ellinor P.T., January C.T., Peterson R.T., Milan D.J. ( 2010). Novel chemical suppressors of long QT syndrome identified by an in vivo functional screen. Circulation [Epub ahead of print] 10.1161/CIRCULATIONAHA.110.003731 [PMC free article] [PubMed] [Cross Ref]


Articles from Disease Models & Mechanisms are provided here courtesy of Company of Biologists