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XB-ART-39247
Biochem Biophys Res Commun 2009 Apr 03;3812:159-64. doi: 10.1016/j.bbrc.2009.02.042.
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Biophysical properties of zebrafish ether-à-go-go related gene potassium channels.

Scholz EP , Niemer N , Hassel D , Zitron E , Bürgers HF , Bloehs R , Seyler C , Scherer D , Thomas D , Kathöfer S , Katus HA , Rottbauer WA , Karle CA .


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The zebrafish is increasingly recognized as an animal model for the analysis of hERG-related diseases. However, functional properties of the zebrafish orthologue of hERG have not been analyzed yet. We heterologously expressed cloned ERG channels in Xenopus oocytes and analyzed biophysical properties using the voltage clamp technique. zERG channels conduct rapidly activating and inactivating potassium currents. However, compared to hERG, the half-maximal activation voltage of zERG current is shifted towards more positive potentials and the half maximal steady-state inactivation voltage is shifted towards more negative potentials. zERG channel activation is delayed and channel deactivation is accelerated significantly. However, time course of zERG conducted current under action potential clamp is highly similar to the human orthologue. In summary, we show that ERG channels in zebrafish exhibit biophysical properties similar to the human orthologue. Considering the conserved channel function, the zebrafish represents a valuable model to investigate human ERG channel related diseases.

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Species referenced: Xenopus
Genes referenced: erg gnao1 kcnh2
GO keywords: potassium channel activity [+]

???displayArticle.disOnts??? short QT syndrome [+]
???displayArticle.omims??? LONG QT SYNDROME 3; LQT3 [+]