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Sox2 is required for sensory organ development in the mammalian inner ear
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Sox2 is required for sensory organ development in the mammalian inner ear
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Journal Article
Sox2 is required for sensory organ development in the mammalian inner ear
2005
Overview
Sensory hair cells and their associated non-sensory supporting cells in the inner ear are fundamental for hearing and balance. They arise from a common progenitor
1
, but little is known about the molecular events specifying this cell lineage. We recently identified two allelic mouse mutants, light coat and circling (
Lcc
) and yellow submarine (
Ysb
), that show hearing and balance impairment
2
.
Lcc
/
Lcc
mice are completely deaf, whereas
Ysb
/
Ysb
mice are severely hearing impaired
2
. We report here that inner ears of
Lcc
/
Lcc
mice fail to establish a prosensory domain and neither hair cells nor supporting cells differentiate, resulting in a severe inner ear malformation, whereas the sensory epithelium of
Ysb
/
Ysb
mice shows abnormal development with disorganized and fewer hair cells. These phenotypes are due to the absence (in
Lcc
mutants) or reduced expression (in
Ysb
mutants) of the transcription factor SOX2, specifically within the developing inner ear. SOX2 continues to be expressed in the inner ears of mice lacking
Math1
(also known as
Atoh1
and
HATH1
), a gene essential for hair cell differentiation, whereas
Math1
expression is absent in
Lcc
mutants, suggesting that
Sox2
acts upstream of
Math1
.
Publisher
Nature Publishing Group UK,Nature Publishing,Nature Publishing Group
Subject
/ Animals
/ Basic Helix-Loop-Helix Transcription Factors
/ Biological and medical sciences
/ Cells
/ DNA-Binding Proteins - deficiency
/ DNA-Binding Proteins - genetics
/ DNA-Binding Proteins - metabolism
/ Fundamental and applied biological sciences. Psychology
/ Hair
/ Hair Cells, Auditory, Inner - abnormalities
/ Hair Cells, Auditory, Inner - metabolism
/ Hair Cells, Auditory, Inner - pathology
/ Humanities and Social Sciences
/ letter
/ Mammals
/ Mice
/ Mutants
/ Nerve Tissue Proteins - deficiency
/ Nerve Tissue Proteins - genetics
/ Nerve Tissue Proteins - metabolism
/ Rodents
/ Science
/ Trans-Activators - deficiency
/ Trans-Activators - metabolism
/ Transcription Factors - deficiency
/ Transcription Factors - genetics
