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Updated: Dec 31, 2025

Posterior Semicircular Canal Approach for Inner Ear Gene Delivery in Neonatal Mouse

Posterior Semicircular Canal Approach for Inner Ear Gene Delivery in Neonatal Mouse

Published on: March 2, 2018

DNAJC14 Ameliorates Inner Ear Degeneration in the DFNB4 Mouse Model.

Hye Ji Choi¹, Hyun Jae Lee¹, Jin Young Choi²

¹Department of Otorhinolaryngology, Yonsei University College of Medicine, Seoul 120-752, Republic of Korea.

Molecular Therapy. Methods & Clinical Development

|January 8, 2020

Summary

This summary is machine-generated.

The His723Arg mutation in SLC26A4 causes genetic hearing loss (DFNB4). DNAJC14 activation rescues pendrin expression and activity, offering a potential therapeutic target for cochlear degeneration.

Keywords:

DFNB4 DNAJC14 H723R Pendred syndrome chaperone flavivirus genetic hearing loss pendrin slc26a4 unconventional secretion

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Modified Experimental Conditions for Noise-Induced Hearing Loss in Mice and Assessment of Hearing Function and Outer Hair Cell Damage

Modified Experimental Conditions for Noise-Induced Hearing Loss in Mice and Assessment of Hearing Function and Outer Hair Cell Damage

Published on: February 10, 2023

Area of Science:

Genetics
Otolaryngology
Molecular Biology

Background:

The His723Arg (H723R) mutation in SLC26A4 is the most common cause of DFNB4, a form of genetic hearing loss in East Asia.
The primary pathology involves a protein-folding defect in pendrin, with no current curative treatments for the associated hearing loss.

Purpose of the Study:

To investigate the potential of activating the chaperonin DNAJC14 to rescue H723R-pendrin expression and activity.
To evaluate the therapeutic potential of DNAJC14 in a mouse model mimicking human DFNB4 pathology.

Main Methods:

In vitro studies involving Japanese encephalitis virus (JEV) to activate DNAJC14 and assess H723R-pendrin rescue.
Generation of human H723R-pendrin transgenic mice on a slc26a4 knockout background.
Crossbreeding transgenic mice with DNAJC14-overexpressing mice to analyze effects on cochlear pathology and physiology.

Main Results:

DNAJC14 activation, particularly via toxin-attenuated JEV, rescued H723R-pendrin surface expression and anion exchange activity in vitro.
DNAJC14 overexpression in mice reduced cochlear hydrops and preserved outer hair cells, with thicker stria vascularis and spiral ligament, and increased KCNJ10 expression.
Despite amelioration of cochlear degeneration, DNAJC14 overexpression did not recover hearing function or reverse enlarged endolymphatic hydrops.

Conclusions:

DNAJC14 overexpression mitigates cochlear degeneration caused by misfolded pendrin in a mouse model of DFNB4.
DNAJC14 represents a potential therapeutic target for ameliorating cochlear pathology in DFNB4, although further research is needed to restore hearing function.