Navigation Links
Gene discovery reveals a critical protein's function in hearing
Date:8/21/2009

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 by University of Iowa and Kansas State University researchers. The findings appear in the August 21 issue of the open-access journal 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.


'/>"/>

Contact: Tamsin Eldred
press@plos.org
44-122-346-3339
Public Library of Science
Source:Eurekalert

Page: 1

Related biology news :

1. Discovery of genetic mutation in Leigh syndrome
2. Discovery to aid in future treatments of third-world parasites
3. New discovery suggests trees evolved camouflage defense against long extinct predator
4. Down Under dinosaur burrow discovery provides climate change clues
5. GUMC discovery highlights new direction for drug discovery
6. Discovery may provide new treatments for alcohol dependence
7. New nanoparticles could revolutionize therapeutic drug discovery
8. Discovery of a water snake that startles fish in a way that makes them flee into its jaws
9. New discovery suggests mammoths survived in Britain until 14,000 years ago
10. Discovery of the cells water gate may lead to new cancer drugs
11. Aussie and Kiwi researchers make double MS genetic discovery
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:6/2/2016)... -- Perimeter Surveillance & Detection Systems, Biometrics ... Support & Other Service  The latest report ... analysis of the global Border Security market . ... $17.98 billion in 2016. Now: In November ... software and hardware technologies for advanced video surveillance. ...
(Date:5/16/2016)... , May 16, 2016   EyeLock LLC , ... announced the opening of an IoT Center of Excellence ... and expand the development of embedded iris biometric applications. ... level of convenience and security with unmatched biometric accuracy, ... identity aside from DNA. EyeLock,s platform uses video technology ...
(Date:4/28/2016)... BANGALORE, India , April 28, 2016 ... of Infosys (NYSE: INFY ), and Samsung SDS, ... partnership that will provide end customers with a more ... payment services.      (Logo: http://photos.prnewswire.com/prnh/20130122/589162 ) ... financial services, but it also plays a fundamental part in ...
Breaking Biology News(10 mins):
(Date:6/27/2016)... 27, 2016  Sequenom, Inc. (NASDAQ: SQNM ... lives through the development of innovative products and services, ... the United States denied its petition to ... of Sequenom,s U.S. Patent No. 6,258,540 (",540 Patent") are ... by the Supreme Court,s Mayo Collaborative Services v. Prometheus ...
(Date:6/27/2016)... June 27, 2016  Liquid Biotech ... funding of a Sponsored Research Agreement with The ... cells (CTCs) from cancer patients.  The funding will ... levels correlate with clinical outcomes in cancer patients ... will then be employed to support the design ...
(Date:6/24/2016)... ... 24, 2016 , ... Researchers at the Universita Politecnica delle Marche in Ancona ... or pleural mesothelioma. Their findings are the subject of a new article on the ... are signposts in the blood, lung fluid or tissue of mesothelioma patients that can ...
(Date:6/23/2016)... SILVER SPRING, Md. , June 23, 2016 ... evidence collected from the crime scene to track the criminal ... sick, and the U.S. Food and Drug Administration (FDA) uses ... Sound far-fetched? It,s not. ... whole genome sequencing to support investigations of foodborne illnesses. Put ...
Breaking Biology Technology: