Circling, head tilt and head tossing behavior; the mutants are deaf.

The mutants show circling and head tossing behavior, and a slight head tilt, observable at 4 weeks of age (+/-1 week; n=18). Two mutants tested for Auditory brainstem responses were found to be deaf. A colony can be maintained through normal breeding. Because of the map position of this mutation, and its phenotypic similarity to Kcne1 mutations (potassium voltage-gated channel, Isk-related subfamily, member 1, see also Vetter et al., 1996), complementation tests with Kcne1^pkr (Kcne1 punk rocker; Letts et al., 2000) have been performed to determine if NMF190 represents an allele of Kcne1. Two litters of heterozygote matings between NMF190 and Kcne1^pkr yielded 2 mutants in a total of 12 progeny, suggesting that NMF190 indeed represents an allele of Kcne1. The NMF190 mutants might therefore be useful for the study of Jervell and Lange-Nielsen syndrome, a human autosomal recessive disorder, affecting auditory and cardiac functions (Letts et al., 2000). Serial sections of the ears of one mutant (32 days of age) showed a thickened tectorial membrane with mineralization and adhesion to Reissner's membrane; whole ear exam of one other mutant (30 days of age) showed abnormally shaped maculae. Standard pathology work-up showed no other abnormalities.

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• Cryopreserved embryos (EM)