In this project, Dr Bo Zhao at Indiana University, USA, explores the role of a protein called CLIC5 in hearing, and how mutations of its gene cause hearing loss.
Project start date: April 2020
Project end date: November 2023
Read about the project outcomes here.
About the project
Mutations in the gene encoding a protein called CLIC5 cause hearing loss by unknown mechanisms. CLIC5 is found in the sound-sensing cells of the inner ear, the inner hair cells, precisely in the hair-like structures, called ‘stereocilia’, which are essential for detecting sound.
We don’t yet understand exactly what CLIC5 does, but we do know that it interacts with another protein called taperin, which has also been linked to hearing loss. This project will investigate why mutations in CLIC5 cause hearing loss.
How it works
The researchers will use different techniques to undertstand the role of CLIC5 in how hair cells form and develop. They will study the interaction between CLIC5 and taperin and its function, and seek to identify other proteins that interact with CLIC5, and investigate what they do in hair cells.
How will this research benefit people at risk of hearing loss?
A detailed understanding of the role of CLIC5 in hearing may help identify new molecules and processes that could be targeted with drugs aiming to correct hearing loss caused by CLIC5 mutations.
The research will serve as a basis for the discovery of other genes which are linked to hearing loss, paving the way for the subsequent discovery of new treatments for hearing loss.
What we’ve learned so far
The researchers studied the function of CLIC5 and successfully identified interactions of CLIC5 with other proteins. This led to the identification of a new gene essential for hearing.
They also tested some putative therapeutic target to restore the hearing loss caused by CLIC5 mutation, which in the future may help develop a way to effectively restore CLIC5 mutation-induced hearing loss.
About the researcher
Dr Bo Zhao is Associate Professor of Otolaryngology in the Head & Neck Surgery at Indiana University, USA. He was awarded an RNID Discovery Research Grant for this project in 2020.
My research goal is to develop new therapeutic treatment for some forms of sensorineural hearing loss, caused by defects in cochlear hair cells.”
