TABLE 3.

Mouse models engineered to study KCNE1 function in the inner ear and heart

Mutant allele of Kcne1	Inner ear phenotype	Cardiac phenotype	Age	Reference
deletion of coding exon, replaced with neo-cassette	deaf, circling, head tossing	not evaluated	(P7, 10, 20, 42), 3,5 and 7mth	Vetter et al., 1996
same allele as reported in Vetter et al., 1996	not evaluated	longer QT interval at slow heart rates and a shorter QT interval at faster heart rates	20 wk	Charpentier et al., 1998
same allele as reported in Vetter et al., 1996	not evaluated	longer QT at slow heart rates and shorter QT at faster heart rates	3 wk and 12 wk	Drici et al., 1998
same allele as reported in Vetter et al., 1996	not evaluated	cardiac arrhythmogenicity	3–6 mth	Balasubramaniam et al., 2003
coding exon of Kcne1 deleted and substituted with a LacZ cassette	not evaluated	no prolonged QT interval at slow or fast heart rates	Neonates and adults	Kupershmidt et al., 1999
Kcne1pkr spontaneous C>T (p.Arg67*)	deaf, circling, head tossing	not evaluated	5wk	Letts et al., 2000

wk, week; mth, month; P, postnatal; LacZ, encoding E. coli β-galactosidase