Sox2 is required for sensory organ development in the mammalian inner ear

Amy E. Kiernan, Anna L. Pelling, Keith K. H. Leung, Anna S. P. Tang, Donald M. Bell, Charles Tease, Robin Lovell-Badge, Karen P. Steel and Kathryn S. E. Cheah ()
Additional contact information
Amy E. Kiernan: University of Nottingham
Anna L. Pelling: The University of Hong Kong
Keith K. H. Leung: The University of Hong Kong
Anna S. P. Tang: The University of Hong Kong
Donald M. Bell: MRC National Institute for Medical Research
Charles Tease: MRC Mammalian Genetics Unit
Robin Lovell-Badge: MRC National Institute for Medical Research
Karen P. Steel: University of Nottingham
Kathryn S. E. Cheah: The University of Hong Kong

Nature, 2005, vol. 434, issue 7036, 1031-1035

Abstract: Abstract 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 progenitor1, 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 impairment2. Lcc/Lcc mice are completely deaf, whereas Ysb/Ysb mice are severely hearing impaired2. 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.

Date: 2005
References: Add references at CitEc
Citations: View citations in EconPapers (1)

Downloads: (external link)
https://www.nature.com/articles/nature03487 Abstract (text/html)
Access to the full text of the articles in this series is restricted.

Related works:
This item may be available elsewhere in EconPapers: Search for items with the same title.

Export reference: BibTeX RIS (EndNote, ProCite, RefMan) HTML/Text

Persistent link: https://EconPapers.repec.org/RePEc:nat:nature:v:434:y:2005:i:7036:d:10.1038_nature03487

Ordering information: This journal article can be ordered from
https://www.nature.com/

DOI: 10.1038/nature03487

Access Statistics for this article

Nature is currently edited by Magdalena Skipper

More articles in Nature from Nature
Bibliographic data for series maintained by Sonal Shukla () and Springer Nature Abstracting and Indexing ().