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Myosin VIIA defects, which underlie the Usher 1B syndrome in humans, lead to deafness in Drosophila.

Publication ,  Journal Article
Todi, SV; Franke, JD; Kiehart, DP; Eberl, DF
Published in: Current biology : CB
May 2005

In vertebrates, auditory and vestibular transduction occurs on apical projections (stereocilia) of specialized cells (hair cells). Mutations in myosin VIIA (myoVIIA), an unconventional myosin, lead to deafness and balance anomalies in humans, mice, and zebrafish; individuals are deaf, and stereocilia are disorganized. The exact mechanism through which myoVIIA mutations result in these inner-ear anomalies is unknown. Proposed inner-ear functions for myoVIIA include anchoring transduction channels to the stereocilia membrane, trafficking stereocilia linking components, and anchoring hair cells by associating with adherens junctions. The Drosophila myoVIIA homolog is crinkled (ck). The Drosophila auditory organ, Johnston's organ (JO), is developmentally and functionally related to the vertebrate inner ear. Both derive from modified epithelial cells specified by atonal and spalt homolog expression, and both transduce acoustic mechanical energy (and references therein). Here, we show that loss of ck/myoVIIA function leads to complete deafness in Drosophila by disrupting the integrity of the scolopidia that transduce auditory signals. We demonstrate that ck/myoVIIA functions to organize the auditory organ, that it is functionally required in neuronal and support cells, that it is not required for TRPV channel localization, and that it is not essential for scolopidial-cell-junction integrity.

Duke Scholars

Published In

Current biology : CB

DOI

EISSN

1879-0445

ISSN

0960-9822

Publication Date

May 2005

Volume

15

Issue

9

Start / End Page

862 / 868

Related Subject Headings

  • Signal Transduction
  • Myosins
  • Myosin VIIa
  • Mutation
  • Microscopy, Confocal
  • Intercellular Junctions
  • Immunohistochemistry
  • Humans
  • Hair Cells, Auditory
  • Evoked Potentials, Auditory
 

Citation

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Todi, S. V., Franke, J. D., Kiehart, D. P., & Eberl, D. F. (2005). Myosin VIIA defects, which underlie the Usher 1B syndrome in humans, lead to deafness in Drosophila. Current Biology : CB, 15(9), 862–868. https://doi.org/10.1016/j.cub.2005.03.050
Todi, Sokol V., Josef D. Franke, Daniel P. Kiehart, and Daniel F. Eberl. “Myosin VIIA defects, which underlie the Usher 1B syndrome in humans, lead to deafness in Drosophila.Current Biology : CB 15, no. 9 (May 2005): 862–68. https://doi.org/10.1016/j.cub.2005.03.050.
Todi SV, Franke JD, Kiehart DP, Eberl DF. Myosin VIIA defects, which underlie the Usher 1B syndrome in humans, lead to deafness in Drosophila. Current biology : CB. 2005 May;15(9):862–8.
Todi, Sokol V., et al. “Myosin VIIA defects, which underlie the Usher 1B syndrome in humans, lead to deafness in Drosophila.Current Biology : CB, vol. 15, no. 9, May 2005, pp. 862–68. Epmc, doi:10.1016/j.cub.2005.03.050.
Todi SV, Franke JD, Kiehart DP, Eberl DF. Myosin VIIA defects, which underlie the Usher 1B syndrome in humans, lead to deafness in Drosophila. Current biology : CB. 2005 May;15(9):862–868.
Journal cover image

Published In

Current biology : CB

DOI

EISSN

1879-0445

ISSN

0960-9822

Publication Date

May 2005

Volume

15

Issue

9

Start / End Page

862 / 868

Related Subject Headings

  • Signal Transduction
  • Myosins
  • Myosin VIIa
  • Mutation
  • Microscopy, Confocal
  • Intercellular Junctions
  • Immunohistochemistry
  • Humans
  • Hair Cells, Auditory
  • Evoked Potentials, Auditory