Tsukushi is essential for the development of the inner ear

Tsukushi (TSK)-a small, secreted, leucine-rich-repeat proteoglycan-interacts with and regulates essential cellular signaling cascades. However, its functions in the mouse inner ear are unknown. In this study, measurement of auditory brainstem responses, fluorescence microscopy, and scanning electron...

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Bibliographic Details
Published in:Molecular brain 2020-03, Vol.13 (1), p.29-29, Article 29
Main Authors: Miwa, Toru, Ohta, Kunimasa, Ito, Naofumi, Hattori, Satoko, Miyakawa, Tsuyoshi, Takeo, Toru, Nakagata, Naomi, Song, Wen-Jie, Minoda, Ryosei
Format: Article
Language:English
Subjects:
Animals
BMP4
Bone Morphogenetic Protein 4 - metabolism
Bone morphogenetic proteins
Ear, Inner - embryology
Ear, Inner - metabolism
Electron microscopy
Embryonic Development
Fluorescence microscopy
Ganglion cysts
Gene Expression Regulation, Developmental
Genes
Hair
Hair cell stereocilia
Hair Cells, Auditory, Inner - metabolism
Hearing
Hearing loss
Inner ear
Ligaments
Ligaments - metabolism
Localization
Mice, Knockout
Microscopy
Morphology
Proteoglycans - deficiency
Proteoglycans - genetics
Proteoglycans - metabolism
Quantitative analysis
Rodents
Signal Transduction
Sox2
SOXB1 Transcription Factors - metabolism
Spiral Ganglion - metabolism
Stereocilia - metabolism
Tsukushi
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Summary:Tsukushi (TSK)-a small, secreted, leucine-rich-repeat proteoglycan-interacts with and regulates essential cellular signaling cascades. However, its functions in the mouse inner ear are unknown. In this study, measurement of auditory brainstem responses, fluorescence microscopy, and scanning electron microscopy revealed that TSK deficiency in mice resulted in the formation of abnormal stereocilia in the inner hair cells and hearing loss but not in the loss of these cells. TSK accumulated in nonprosensory regions during early embryonic stages and in both nonprosensory and prosensory regions in late embryonic stages. In adult mice, TSK was localized in the organ of Corti, spiral ganglion cells, and the stria vascularis. Moreover, loss of TSK caused dynamic changes in the expression of key genes that drive the differentiation of the inner hair cells in prosensory regions. Finally, our results revealed that TSK interacted with Sox2 and BMP4 to control stereocilia formation in the inner hair cells. Hence, TSK appears to be an essential component of the molecular pathways that regulate inner ear development.
ISSN:1756-6606
1756-6606
DOI:10.1186/s13041-020-00570-z