Researchers at the University of Iowa and Kansas State University say a deafness-causing gene defect in mice helped identify a new protein that protects sensory cells in the ear, according to findings in PLoS Genetics.
In humans, hereditary deafness is one of the most common birth defects, yet most genes involved in hearing are unidentified. Mice are used as research models because mouse and human auditory genetics are very similar.
Using a deaf mouse model generated at The Jackson Laboratory, the team identified the deafness-causing defect in the claudin-9 gene. The mutated gene fails to produce normal claudin-9 protein, which, the UI team showed, is needed to maintain the proper distribution of potassium in the inner ear.
"Genes in the claudin family number at least 24 and produce proteins that prevent ions, including potassium, from moving between cells," said senior author Botond Banfi. "Sensory cells in the hearing organ are bathed in a high potassium solution on one side and in a low potassium solution on the other side. We found that claudin-9 is very important in keeping the amount of potassium on the two sides separate. This separation protects sensory cells from potassium intoxication."
When claudin-9 is mutated, potassium floods the wrong part of the sensory cells, killing most and leaving the remaining ones functionally defective.
In follow-up efforts, Banfi and colleagues are screening people with hearing impairment to see if some of them have a mutation in claudin-9.
FINANCIAL DISCLOSURE: The study was funded in part by a grant from the National Institutes of Health and support from the UI Roy J. and Lucille A. Carver College of Medicine and the UI Gene Therapy Center. The funders had no role in study design, data collection and analysis, decision to publish, or preparation of the manuscript.
COMPETING INTERESTS: The authors have declared that no competing interests exist.
CITATION: Nakano Y, Kim SH, Kim H-M, Sanneman JD, Zhang Y, et al. (2009) A Claudin-9–Based Ion Permeability Barrier Is Essential for Hearing. PLoS Genet 5(8): e1000610. doi:10.1371/journal.pgen.1000610
- PHYSICAL SCIENCES
- EARTH SCIENCES
- LIFE SCIENCES
- SOCIAL SCIENCES
Subscribe to the newsletter
Stay in touch with the scientific world!
Know Science And Want To Write?
- Who Is Trying To Destroy The Internet?
- Debunking: A President Of The US Could Order A Nuclear Attack At A Moments Notice On A Whim
- A Dimuon Particle At 30 GeV In ALEPH ??
- DDoS War: How Zombie Fridges Bit The Internet In The a$$ Today.
- President Obama, Why Humans On Mars Right Now Are Bad For Science
- Biofuels Are A Climate Mistake
- Hearing Voices Again? It's More Common Than You Think
- "And of course NIBIRU . Nibirumaster..."
- "THEN robert I should be concerned about the asteroid 2000 ET70 ????..."
- "I urge you to study Pribams Holonomic mind theory, as I believe it fits your query quite well here..."
- "NASA rarely comment on stories like this. It's not their mission to debunk doomsday hoaxes. They..."
- "Ok fair point and this is 2 separate questions 1 what do nasa at this moment in time think of nibiru..."
- To Avoid Adult Dysfunction Start 'IN UTERO'
- ACSH Medical Director Named One Of America's Top Pediatricians, We're In The Economist, And More
- The Math of Hunting and Fishing: When to Work Together
- Placebo: Bubbles Of Nothing Are Still Not Something
- People Who Take Drugs May Be Likelier to Commit Suicide
- Improved 'Screen Time' Guidelines Could Make Parents & Kids Happier