The ubiquitin-proteasome system in normal hearing and deafness.

[en] Post-translational modifications of proteins are essential for the proper development and function of many tissues and organs, including the inner ear. Ubiquitination is a highly selective post-translational modification that involves the covalent conjugation of ubiquitin to a substrate protein. The most common outcome of protein ubiquitination is degradation by the ubiquitin-proteasome system (UPS), preventing the accumulation of misfolded, damaged, and excess proteins. In addition to proteasomal degradation, ubiquitination regulates other cellular processes, such as transcription, translation, endocytosis, receptor activity, and subcellular localization. All of these processes are essential for cochlear development and maintenance, as several studies link impairment of UPS with altered cochlear development and hearing loss. In this review, we provide insight into the well-oiled machinery of UPS with a focus on its confirmed role in normal hearing and deafness and potential therapeutic strategies to prevent and treat UPS-associated hearing loss.

Disciplines :

Biochemistry, biophysics & molecular biology

Author, co-author :

Pouyo, Lionel Ronald ; Université de Liège - ULiège > GIGA > GIGA Stem Cells - Developmental Neurobiology

Chung, Keshi ; Université de Liège - ULiège > Département des sciences cliniques > Neurologie

Delacroix, Laurence ; Université de Liège - ULiège > GIGA > GIGA Stem Cells - Developmental Neurobiology

Malgrange, Brigitte ; Université de Liège - ULiège > GIGA > GIGA Neurosciences

Language :

English

Title :

The ubiquitin-proteasome system in normal hearing and deafness.

Publication date :

29 September 2021

Journal title :

Hearing Research

ISSN :

0378-5955

eISSN :

1878-5891

Publisher :

Elsevier B.V., Netherlands

Pages :

108366

Peer reviewed :

Peer Reviewed verified by ORBi

Additional URL :

https://api.elsevier.com/content/article/PII:S0378595521002008?httpAccept=text/xml

Funding text :

This work was supported by grants from the Belgian National Funds for Scientific Research (FSR-FNRS, Belgium), the Fondation Léon Frédéricq (ULiege, Belgium) and the Fonds Speciaux (ULiege, Belgium).This work was supported by grants from the Belgian National Funds for Scientific Research (FSR-FNRS, Belgium), the Fondation Léon Frédéricq (ULiege, Belgium) and the Fonds Spéciaux (ULiege, Belgium).

Available on ORBi :

since 04 April 2022

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