Hearing Restored to Mice with Genetic Deafness through Gene Therapy

Hearing Restored to Mice with Genetic Deafness through Gene Therapy

Posted by Leanne Kodsman on

After focusing on the transmembrane channel-like protein 1 (TMC1) gene, researchers at Boston Children's Hospital and Harvard Medical School were able to restore hearing in mice with deafness caused through genetic disorders using gene therapy.

According to GEN News, "TMC1 forms a channel that allows calcium to enter the cell when moved by sound waves, including an electrical signal that is sent to the brain and interpreted as an auditory signal." Close to 8% of genetic deafness cases are the result of TMC1 issues.


After focusing on the transmembrane channel-like protein 1 (TMC1) gene, researchers at Boston Children's Hospital and Harvard Medical School were able to restore hearing in mice with deafness caused through genetic disorders using gene therapy.

According to GEN News, "TMC1 forms a channel that allows calcium to enter the cell when moved by sound waves, including an electrical signal that is sent to the brain and interpreted as an auditory signal." Close to 8% of genetic deafness cases are the result of TMC1 issues.

While not yet ready for clinical trials, senior author Jeffrey Holt, Ph.D., associate professor of otolaryngology explains that this type of gene therapy could be developed for use in humans.

Along with his colleagues, Dr. Holt designed a virus to contain a working copy of TMC1 and a promoter sequence, to make sure that the gene was turned on correctly. This was injected into the inner ear of two different mouse strains. In the first, TMC1 was completely deleted and in the second, the Beethoven mice, a mutation was present where TMC1 was rendered ineffective.

In order to test the hearing of mice that received the virus, scientists placed them in a "startle box," where loud tones were sounded and the reaction of the mice were measured and observed through a force plate on the floor below them. Surprisingly, both strains of mice were able to detect the auditory signals.

Researchers also observed that the mice who had received gene therapy were able to hear addition sounds in the 80-decibel range.

Dr. Holt's hope is to start clinical trials of TMC1 gene therapy within 5 to 10 years through a partnership with Boston Children's department of otolaryngology.


    Innovative Research was established in 1998 after the realization that dependable, high-quality, and affordable research materials were hard to come by. Starting with core products like human plasma and serum, Innovative Research has grown to be a trusted supplier of all lab reagents, including human biologicals and ELISA kits. Today, we manufacture and supply thousands of high-quality human and animal biologicals including plasma, serum, tissues, and proteins.


Related Posts

In Case You Missed It ... Reference Roundup!
The next big thing: it's what you do. The biological research materials you need to dis...
Read the Article
Improving Diabetes Treatment: Using Drug-Loaded Nanostructures for Oral Delivery
Did you know? For this study, Single Donor Human Whole Blood was obtained commercially ...
Read the Article
In Case You Missed It ... Reference Roundup for July 3, 2018!
The next big thing: it's what you do. The biological research materials you need to dis...
Read the Article

← Older Post Newer Post →