• heart conduction disease

This report describes a fly model of cardiac arrhythmia and seizure sensitivity using alleles of the fly gene sei, which encodes a voltage-gated potassium channel. Dmel\sei is orthologous to the several members of the 'subfamily H' potassium voltage-gated channel genes in human; it is most closely related to KCNH2, KCNH6, and KCNH7. The human gene KCNH2 is implicated in several forms of cardiac arrhythmia (long QT syndrome 2, MIM:613688, FBhh0000735; short QT syndrome 1, MIM:609620); both exhibit autosomal dominant inheritance. For the Dmel\sei gene, classical loss-of-function alleles, RNAi targeting constructs, and alleles caused by insertional mutagenesis have been generated.

One of the related human genes, Hsap\KCNH7, has been introduced into flies, but has not been characterized.

Two homozygous loss-of-function alleles of Dmel\sei are temperature-sensitive: at elevated temperatures they exhibit paralysis and/or seizures, bradycardia, electrical arrhythmia, and altered myofibrillar structure. Sleep deprivation has been found to increase the seizure susceptibility. Genetic interactions of Dmel\sei have been described; see the sei gene report.

[updated Feb. 2018 by FlyBase; FBrf0222196]