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

Sokol V Todi; Josef D Franke; Daniel P Kiehart; Daniel F Eberl

doi:10.1016/j.cub.2005.03.050

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

Journal article

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

Sokol V Todi, Josef D Franke, Daniel P Kiehart and Daniel F Eberl

Current biology, Vol.15(9), pp.862-868

05/10/2005

DOI: 10.1016/j.cub.2005.03.050

PMCID: PMC1808204

PMID: 15886106

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Abstract

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.

Immunohistochemistry

Deafness - genetics

Humans

Electrophysiology

Signal Transduction - genetics

Mutation - genetics

Myosins - genetics

Drosophila - physiology

Microscopy, Confocal

Ear, Inner - metabolism

Animals

Hair Cells, Auditory - physiology

Cloning, Molecular

Evoked Potentials, Auditory - physiology

Intercellular Junctions - physiology

Dyneins - genetics

Drosophila - genetics

Details

Title: Subtitle: Myosin VIIA defects, which underlie the Usher 1B syndrome in humans, lead to deafness in Drosophila
Creators: Sokol V Todi - Neuroscience Graduate Program, Department of Biological Sciences, The University of Iowa, Iowa City, Iowa 52242, USA
Josef D Franke
Daniel P Kiehart
Daniel F Eberl
Resource Type: Journal article
Publication Details: Current biology, Vol.15(9), pp.862-868
Publisher: England
DOI: 10.1016/j.cub.2005.03.050
PMID: 15886106
PMCID: PMC1808204
ISSN: 0960-9822
eISSN: 1879-0445
Grant note: T32 GM007184 / NIGMS NIH HHS GM33830 / NIGMS NIH HHS GM07184 / NIGMS NIH HHS DC04848 / NIDCD NIH HHS R01 DC004848 / NIDCD NIH HHS R01 GM033830 / NIGMS NIH HHS
Language: English
Date published: 05/10/2005
Academic Unit: Iowa Neuroscience Institute; Biology
Record Identifier: 9984070418002771

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