Navigation Links
Hearing protein required to convert sound into brain signals
Date:6/17/2014

HEIDELBERG, 17 June 2014 A specific protein found in the bridge-like structures that make up part of the auditory machinery of the inner ear is essential for hearing. The absence of this protein or impairment of the gene that codes for this protein leads to profound deafness in mice and humans, respectively, reports a team of researchers in the journal EMBO Molecular Medicine.

"The goal of our study was to identify which isoform of protocadherin-15 forms the tip-links, the essential connections of the auditory mechanotransduction machinery within mature hair cells that are needed to convert sound into electrical signals," remarks Christine Petit, the lead author of the study and Professor at the Institut Pasteur in Paris and at Collge de France.

Three types of protocadherin-15 are known to exist in auditory sensory cells of the inner ear but it was not clear which of these protein isoforms was essential for hearing. "Our work pinpoints the CD2 isoform of protocadherin-15 as an essential component of the tip-link and reveals that the absence of protocadherin-15 CD2 in mouse hair cells results in profound deafness."

Within the hair bundle, the sensory antenna of auditory sensory cells, the tip-link is a bridge-like structure that when stretched can activate the ion channel responsible for generating electrical signals from sound. Tension in the tip-link created by sound stimulation opens this channel of unknown molecular composition thus generating electrical signals and, ultimately, the perception of sound.

The researchers engineered mice that lack only the CD2 isoform of protocadherin-15 exclusively during adulthood. While the absence of this isoform led to profound deafness, the lack of the other protocadherin-15 isoforms in mice did not affect their hearing.

Patients who carry a mutation in the gene encoding protocadherin15 are affected by a rare devastating disorder, Usher syndrome, which is characterized by profound deafness, balance problems and gradual visual loss due to retinitis pigmentosa. In a separate approach, the scientists also sequenced the genes of 60 patients who had profound deafness without balance and visual impairment. Three of these patients were shown to have mutations specifically affecting protocadherin-15 CD2. "The demonstration of a requirement for protocadherin-15 CD2 for hearing not only in mice but also in humans constitutes a major step in the objective of deciphering the components of the auditory mechanotransduction machinery. This isoform can be used as a starting point to identify the other components of the auditory machinery. By focusing our attention on the CD2 isoform of protocadherin-15, we can now consider developing gene therapy strategies for deafness caused by defects in this gene," says EMBO Member Christine Petit.


'/>"/>
Contact: Barry Whyte
barry.whyte@embo.org
European Molecular Biology Organization
Source:Eurekalert  

Related biology news :

1. Scientists test hearing in Bristol Bay beluga whale population
2. New idea for hearing improvement in patients with hearing aids under background noise
3. Understanding hearing
4. Identifying a mystery channel crucial for hearing
5. Now hear this: Scientists discover compound to prevent noise-related hearing loss
6. Cellular channels vital for hearing identified
7. Study: Resveratrol shows promise to protect hearing, cognition
8. UI researcher learns mechanism of hearing is similar to car battery
9. Dolphin hearing system component found in insects
10. USF researchers identify gene mutation linked to old age hearing loss
11. Hearing the telltale sounds of dangerous chemicals
Post Your Comments:
*Name:
*Comment:
*Email:
Related Image:
Hearing protein required to convert sound into brain signals
(Date:5/12/2016)... , May 12, 2016 WearablesResearch.com , ... published the overview results from the Q1 wave of ... recent wave was consumers, receptivity to a program where ... with a health insurance company. "We were ... share," says Michael LaColla , CEO of Troubadour ...
(Date:5/3/2016)... May 3, 2016  Neurotechnology, a provider of ... MegaMatcher Automated Biometric Identification System (ABIS) , a ... projects. MegaMatcher ABIS can process multiple complex biometric ... combination of fingerprint, face or iris biometrics. It ... and MegaMatcher Accelerator , which have ...
(Date:4/28/2016)... 2016 First quarter 2016:   ... with the first quarter of 2015 The gross margin ... (loss: 18.8) and the operating margin was 40% (-13) ... Cash flow from operations was SEK 249.9 M (21.2) , ... unchanged, SEK 7,000-8,500 M. The operating margin for 2016 ...
Breaking Biology News(10 mins):
(Date:6/27/2016)... , June 27, 2016   Ginkgo Bioworks ... to industrial engineering, was today awarded as one ... selection of the world,s most innovative companies. Ginkgo ... scale for the real world in the nutrition, ... engineers work directly with customers including Fortune 500 ...
(Date:6/24/2016)... June 24, 2016 Epic Sciences unveiled ... cancers susceptible to PARP inhibitors by targeting homologous ... (CTCs). The new test has already been incorporated ... multiple cancer types. Over 230 clinical ... response pathways, including PARP, ATM, ATR, DNA-PK and ...
(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)... ... 2016 , ... UAS LifeSciences, one of the leading manufacturers ... Probiotics, into Target stores nationwide. The company, which has been manufacturing high quality ... list of well-respected retailers. This list includes such fine stores as Whole Foods, ...
Breaking Biology Technology: