The mutants are small and exhibit an unsteady gait, which can be detected at 4 weeks of age (+/- 0.48; n=21). A colony can be maintained through heterozygote matings. Two female mutants tested showed weights slightly (approximately 10%) below normal. Two mutants were tested for Fear-potentiated-startle and Acoustic startle response, and for each screen one mutant showed abnormal, one normal results. Unaffected littermates showed normal results (n=5). Similarly, auditory brainstem responses (at 6 weeks of age) showed one mutant to be deaf and one to be severely hearing impaired (only a late response to a 8kHz stimulus at 90db was observed), while two unaffected littermates tested normal.
<p>Standard pathology work-up on 3 mutants (113 - 161 days of age) showed dystrophic axons and a few abnormal muscle fibers. Large lobules of lymphocytes in the lung and mild hydrocephaly were also present in one mutant.  Retinal degeneration, and a loss of hair cells, supporting cells and ganglionic cells of the ear were also observed in the oldest mutant. 



These mice carry an ENU-induced mutation characterized by dystrophic axons and abnormal muscle fibers.

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