Channelpedia

PubMed 8994043


Referenced in: none

Automatically associated channels: Kv10.1 , Kv11.1



Title: The seizure locus encodes the Drosophila homolog of the HERG potassium channel.

Authors: X J Wang, E R Reynolds, P Déak, L M Hall

Journal, date & volume: J. Neurosci., 1997 Feb 1 , 17, 882-90

PubMed link: http://www.ncbi.nlm.nih.gov/pubmed/8994043


Abstract
Mutations in the seizure (sei) locus cause temperature-induced hyperactivity, followed by paralysis. Gene cloning studies have established that the seizure gene product is the Drosophila homolog of HERG, a member of the eag family of K+ channels implicated in one form of hereditary long QT syndrome in humans. A series of five null alleles with premature stop codons are all recessive, but viable. A missense mutation in the sei gene, which changes the charge at a conserved glutamate residue near the outer mouth of the pore, has a semidominant phenotype, suggesting that the mutant seizure protein acts as a poison in a multimeric complex. Transformation rescue of a null allele with a cDNA under the control of an inducible promoter demonstrates that induced expression of seizure potassium channels in adults rescues the paralytic phenotype. This rescue decays with a t1/2 of approximately 1-1.5 d after gene induction is discontinued, providing the first estimate of ion channel stability in an intact, multicellular animal.