Lhx3, a LIM domain transcription factor, is regulated by Pou4f3 in the auditory but not in the vestibular system

Ronna Hertzano, Amiel A. Dror, Mireille Montcouquiol, Zubair M. Ahmed, Buffy Ellsworth, Sally Camper, Thomas B. Friedman, Matthew W. Kelley, Karen B. Avraham
European Journal of Neuroscience. 2007-02-26; 25(4): 999-1005
DOI: 10.1111/j.1460-9568.2007.05332.x

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1. Eur J Neurosci. 2007 Feb;25(4):999-1005.

Lhx3, a LIM domain transcription factor, is regulated by Pou4f3 in the auditory
but not in the vestibular system.

Hertzano R(1), Dror AA, Montcouquiol M, Ahmed ZM, Ellsworth B, Camper S, Friedman
TB, Kelley MW, Avraham KB.

Author information:
(1)Department of Human Molecular Genetics and Biochemistry, Sackler School of
Medicine, Tel Aviv University, Tel Aviv 69978, Israel.

A dominant mutation of the gene encoding the POU4F3 transcription factor
underlies human non-syndromic progressive hearing loss DFNA15. Using
oligonucleotide microarrays to generate expression profiles of inner ears of
Pou4f3(ddl/ddl) mutant and wild-type mice, we have identified and validated Lhx3,
a LIM domain transcription factor, as an in vivo target gene regulated by Pou4f3.
Lhx3 is a hair cell-specific gene expressed in all hair cells of the auditory and
vestibular system as early as embryonic day 16. The level of Lhx3 mRNA is greatly
reduced in the inner ears of embryonic Pou4f3 mutant mice. Our data also show
that the expression of Lhx3 is regulated differently in auditory and vestibular
hair cells. This is the first example of a hair cell-specific gene expressed both
in auditory and in vestibular hair cells, with differential regulation of
expression in these two closely related systems.

DOI: 10.1111/j.1460-9568.2007.05332.x
PMID: 17331196 [Indexed for MEDLINE]


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