Jxc1/Sobp, encoding a nuclear zinc finger protein, is critical for cochlear growth, cell fate, and patterning of the organ of corti.
Journal of Neuroscience. 2008-06-25; 28(26): 6633-6641
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1. J Neurosci. 2008 Jun 25;28(26):6633-41. doi: 10.1523/JNEUROSCI.1280-08.2008.
Jxc1/Sobp, encoding a nuclear zinc finger protein, is critical for cochlear
growth, cell fate, and patterning of the organ of corti.
Chen Z(1), Montcouquiol M, Calderon R, Jenkins NA, Copeland NG, Kelley MW,
(1)Sections on Neurogenetics, Laboratory of Molecular Biology, National Institute
on Deafness and Other Communication Disorders, National Institutes of Health,
Rockville, Maryland 20850, USA.
The mouse cochlea emerges from the ventral pole of the otocyst to form a one and
three-quarter coil. Little is known about the factors that control the growth of
the cochlea. Jackson circler (jc) is a recessive mutation causing deafness
resulting from a growth arrest of the cochlea duct at day 13.5 of embryonic
development. Here, we identify the vertebrate homolog of the Drosophila Sobp
(sine oculis-binding protein) gene (named Jxc1) in the jc locus. Jxc1 encodes a
nuclear protein that has two FCS-type zinc finger domains (PS51024) and bears
nuclear localization signals and highly conserved sequence motifs. Transiently
expressed wild-type protein is targeted to the nucleus, but mutant isoforms were
mislocalized in the cytoplasm. In jc mutants, the cellular patterning of the
organ of Corti is severely disrupted, exhibiting supernumerary hair cells at the
apex, showing mirror-image duplications of tunnel of Corti and inner hair cells,
and expressing ectopic vestibular-like hair cells within Kölliker’s organ. Jxc1
mRNA was detected in inner ear sensory hair cells, supporting cells, and the
acoustic ganglia. Expression was also found in the developing retina, olfactory
epithelium, trigeminal ganglion, and hair follicles. Collectively, our data
support a role for Jxc1 in controlling a critical step in cochlear growth, cell
fate, and patterning of the organ of Corti.
PMID: 18579736 [Indexed for MEDLINE]