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Culture of Embryonic Mouse Cochlear Explants and Gene Transfer by Electroporation
Published on: January 12, 2015
Cochlear function in mice with only one copy of the prestin gene
M A Cheatham1, J Zheng, K H Huynh
1Department of Communication Sciences and Disorders, Northwestern University, Evanston, IL 60208-3550, USA. m-cheatham@northwestern.edu
The Journal of Physiology
|September 17, 2005
Summary
Heterozygous prestin gene deletion does not impair hearing. One gene copy compensates for the loss, maintaining cochlear function and outer hair cell electromotility in mice.
Area of Science:
- Auditory Neuroscience
- Molecular Biology
- Genetics
Background:
- Targeted prestin gene deletion impacts cochlear sensitivity and outer hair cell (OHC) function.
- Previous studies reported hearing deficits in F2 generation heterozygotes.
Purpose of the Study:
- To re-evaluate cochlear function in later generations (F3-F5) of prestin heterozygote mice.
- To investigate the compensatory mechanisms for deleted prestin gene copies.
Main Methods:
- In vivo and in vitro electrophysiology (compound action potential thresholds, tuning curves, cochlear microphonic).
- Non-linear capacitance measurements in isolated OHCs.
- Molecular biology techniques (immunocytochemistry, Western blot for prestin protein; mRNA analysis).
Main Results:
- F3-F5 heterozygote mice showed similar hearing sensitivity and frequency selectivity to wildtype controls.
- OHC somatic electromotility, charge density, and voltage dependence were indistinguishable between genotypes.
- Prestin protein levels were near normal, while mRNA levels were reduced in heterozygotes.
Conclusions:
- A single functional prestin gene copy is sufficient to maintain normal peripheral hearing.
- Heterozygous mice do not exhibit hearing impairment due to compensatory mechanisms.
- Prestin gene dosage does not directly correlate with cochlear sensitivity in later generations.
