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Citation
Cosetti, M., Culang, D., Kotla, S., O'Brien, P., Eberl, D.F., Hannan, F. (2008). Unique transgenic animal model for hereditary hearing loss.  Ann. Otol. Rhinol. Laryngol. 117(11): 827--833.
FlyBase ID
FBrf0206525
Publication Type
Research paper
Abstract

This study capitalizes on the unique molecular and developmental similarities between the auditory organs of Drosophila and mammals, to investigate genes implicated in human syndromic and nonsyndromic hearing loss in a genetically tractable experimental animal model, the fruit fly Drosophila.The Drosophila counterparts of 3 human deafness genes (DIAPH1/DFNA1, ESPN/DFNB36, and TMHS/DF-NB67) were identified by sequence similarity. An electrophysiological assay was used to record sound-evoked potentials in response to an acoustic stimulus, the Drosophila courtship song.Flies with mutations affecting the diaphanous,forked, and CG12026/TMHS genes displayed significant reductions in the amplitude of sound-evoked potentials compared to wild-type flies (p < 0.05 to p < 0.005). The mean responses were reduced from approximately 500 to 600 microV in wild-type flies to approximately 100 to 300 microV in most mutant flies.The identification of significant auditory dysfunction in Drosophila orthologs of human deafness genes will facilitate exploration of the molecular biochemistry of auditory mechanosensation. This may eventually allow for novel diagnostic and therapeutic approaches to human hereditary hearing loss.

PubMed ID
PubMed Central ID
PMC3409696 (PMC) (EuropePMC)
DOI
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    Language of Publication
    English
    Additional Languages of Abstract
    Parent Publication
    Publication Type
    Journal
    Abbreviation
    Ann. Otol. Rhinol. Laryngol.
    Title
    The Annals of otology, rhinology, and laryngology
    ISBN/ISSN
    0003-4894
    Data From Reference
    Aberrations (2)
    Alleles (4)
    Genes (4)
    Human Disease Models (3)
    Insertions (1)