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Gleason, M.R.* ; Nagiel, A.* ; Jamet, S.* ; Vologodskaia, M.* ; López-Schier, H.* ; Hudspeth, A.J.*

The transmembrane inner ear (Tmie) protein is essential for normal hearing and balance in the zebrafish.

Proc. Natl. Acad. Sci. U.S.A. 106, 21347-2152 (2009)
DOI PMC
Open Access Gold as soon as Publ. Version/Full Text is submitted to ZB.
Little is known about the proteins that mediate mechanoelectrical transduction, the process by which acoustic and accelerational stimuli are transformed by hair cells of the inner ear into electrical signals. In our search for molecules involved in mechanotransduction, we discovered a line of deaf and uncoordinated zebrafish with defective hair-cell function. The hair cells of mutant larvae fail to incorporate fluorophores that normally traverse the transduction channels and their ears lack microphonic potentials in response to vibratory stimuli. Hair cells in the posterior lateral lines of mutants contain numerous lysosomes and have short, disordered hair bundles. Their stereocilia lack two components of the transduction apparatus, tip links and insertional plaques. Positional cloning revealed an early frameshift mutation in tmie, the zebrafish ortholog of the mammalian gene transmembrane inner ear. The mutant line therefore affords us an opportunity to investigate the role of the corresponding protein in mechanoelectrical transduction.
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Publication type Article: Journal article
Document type Scientific Article
Language english
Publication Year 2009
HGF-reported in Year 0
ISSN (print) / ISBN 0027-8424
e-ISSN 1091-6490
Quellenangaben Volume: 106, Issue: 50, Pages: 21347-2152 Article Number: , Supplement: ,
Publisher National Academy of Sciences
Reviewing status Peer reviewed
PubMed ID 19934034
Erfassungsdatum 2009-12-31