Drosophila and mammals contain a family of three related voltage-gated potassium channel genes: eag, erg, and elk. Similar in structure to the Kv-class of voltage-gated potassium channels, these channels have the six-membrane-spanning architecture. Mutations in the erg gene underlie a human genetic disease known as long QT syndrome, which produces arrhythmias and an increased incidence of sudden death. Mutations in the erg gene may increase susceptibility to arrhythmia by prolonging the cardiac action potential. The erg gene family consists of three genes: erg1, erg2, and erg3. Abundant nervous system expression of erg1 indicates that it may be involved in nervous system function. However, erg1 is also expressed in non-neural tissues. On the other hand, erg2 and erg3 genes are expressed exclusively in the nervous system. Expression of the erg2 gene is more limited and is located primarily in the prevertebral ganglia. Although the physiological role of the erg channels is unknown, each channel produces a different current that may differentially contribute to the development of long QT syndrome.
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