Article (Scientific journals)
The ubiquitin-proteasome system in normal hearing and deafness.
Pouyo, Lionel Ronald; Chung, Keshi; Delacroix, Laurence et al.
2021In Hearing Research, p. 108366
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Keywords :
ARHL; Cochlea; Deafness; Development; UPS; Sensory Systems
Abstract :
[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
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).
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