Jackson circler mice have a recessive mutation that causes deafness and circling behavior due to defective development of the inner ear. Chen et al. have identified the gene containing this mutation, Jxc1, and report that elongation and patterning of the cochlea was greatly disrupted in mutants.
Fewer rows of hair cells were present in some regions, whereas extra rows appeared in others. In addition, mutant cochlea had a mirror-image duplication of the tunnel of Corti and inner hair cells, and ectopic, vestibular-like hair cells were present.
The vestibular system was also abnormal: the utricles and saccules were significantly thicker in mutants, and their surface areas were smaller. The sequence of Jxc1 indicates that it has two phenylalanine-cysteine-serine-type zinc-finger domains, suggesting that it is a transcriptional silencing factor.
The protein is normally expressed in the nucleus, but the mutant protein, which is a truncated form, also appears in the cytoplasm.-Society for Neuroscience
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