Featured Research

from universities, journals, and other organizations

Gene Discovery Reveals Critical Protein's Function In Hearing

Date:
August 25, 2009
Source:
University of Iowa
Summary:
Discovery of a deafness-causing gene defect in mice has helped identify a new protein that protects sensory cells in the ear, according to a new study. The mutated gene fails to produce normal claudin-9 protein, which, the scientists showed, is needed to maintain the proper distribution of potassium in the inner ear.

When claudin-9 is mutated, potassium floods the wrong part of the ear's sensory cells, killing a layer of them, as indicated by the arrow in this image.
Credit: University of Iowa/PLoS Genetics

Discovery of a deafness-causing gene defect in mice has helped identify a new protein that protects sensory cells in the ear, according to a study led by University of Iowa researchers. The findings, which also involved Kansas State University, appear in the Aug. 21 issue of the open-access journal PLoS Genetics.

Related Articles


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 University of Iowa 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 the paper's senior author Botond Banfi, M.D., Ph.D., assistant professor of anatomy and cell biology in the University of Iowa Carver College of Medicine. "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, including the study's first author Yoko Nakano, Ph.D., University of Iowa assistant research scientist in anatomy and cell biology, are screening people with hearing impairment to see if some of them have a mutation in claudin-9.


Story Source:

The above story is based on materials provided by University of Iowa. Note: Materials may be edited for content and length.


Journal Reference:

  1. Nakano et al. A Claudin-9-Based Ion Permeability Barrier Is Essential for Hearing. PLoS Genetics, 2009; 5 (8): e1000610 DOI: 10.1371/journal.pgen.1000610

Cite This Page:

University of Iowa. "Gene Discovery Reveals Critical Protein's Function In Hearing." ScienceDaily. ScienceDaily, 25 August 2009. <www.sciencedaily.com/releases/2009/08/090821135100.htm>.
University of Iowa. (2009, August 25). Gene Discovery Reveals Critical Protein's Function In Hearing. ScienceDaily. Retrieved November 28, 2014 from www.sciencedaily.com/releases/2009/08/090821135100.htm
University of Iowa. "Gene Discovery Reveals Critical Protein's Function In Hearing." ScienceDaily. www.sciencedaily.com/releases/2009/08/090821135100.htm (accessed November 28, 2014).

Share This


More From ScienceDaily



More Health & Medicine News

Friday, November 28, 2014

Featured Research

from universities, journals, and other organizations


Featured Videos

from AP, Reuters, AFP, and other news services

Rural India's Low-Cost Sanitary Pad Revolution

Rural India's Low-Cost Sanitary Pad Revolution

AFP (Nov. 28, 2014) — One man hopes his invention -– a machine that produces cheap sanitary pads –- will help empower Indian women. Duration: 01:51 Video provided by AFP
Powered by NewsLook.com
Research on Bats Could Help Develop Drugs Against Ebola

Research on Bats Could Help Develop Drugs Against Ebola

AFP (Nov. 28, 2014) — In Africa's only biosafety level 4 laboratory, scientists have been carrying out experiments on bats to understand how virus like Ebola are being transmitted, and how some of them resist to it. Duration: 01:18 Video provided by AFP
Powered by NewsLook.com
WHO Says Male Ebola Survivors Should Abstain From Sex

WHO Says Male Ebola Survivors Should Abstain From Sex

Newsy (Nov. 28, 2014) — WHO cites four studies that say Ebola can still be detected in semen up to 82 days after the onset of symptoms. Video provided by Newsy
Powered by NewsLook.com
Ebola Leaves Orphans Alone in Sierra Leone

Ebola Leaves Orphans Alone in Sierra Leone

AFP (Nov. 27, 2014) — The Ebola epidemic sweeping Sierra Leone is having a profound effect on the country's children, many of whom have been left without any family members to support them. Duration: 01:02 Video provided by AFP
Powered by NewsLook.com

Search ScienceDaily

Number of stories in archives: 140,361

Find with keyword(s):
 
Enter a keyword or phrase to search ScienceDaily for related topics and research stories.

Save/Print:
Share:  

Breaking News:

Strange & Offbeat Stories

 

Health & Medicine

Mind & Brain

Living & Well

In Other News

... from NewsDaily.com

Science News

Health News

Environment News

Technology News



Save/Print:
Share:  

Free Subscriptions


Get the latest science news with ScienceDaily's free email newsletters, updated daily and weekly. Or view hourly updated newsfeeds in your RSS reader:

Get Social & Mobile


Keep up to date with the latest news from ScienceDaily via social networks and mobile apps:

Have Feedback?


Tell us what you think of ScienceDaily -- we welcome both positive and negative comments. Have any problems using the site? Questions?
Mobile iPhone Android Web
Follow Facebook Twitter Google+
Subscribe RSS Feeds Email Newsletters
Latest Headlines Health & Medicine Mind & Brain Space & Time Matter & Energy Computers & Math Plants & Animals Earth & Climate Fossils & Ruins