Molecular Biomedicine· 2026Q1· Review
The ubiquitin–proteasome system and proteostasis in the cochlea and sensorineural hearing disorders
- 0citations
- Q1SCImago
- 2026year
Short summary
This review highlights the ubiquitin-proteasome system (UPS) as a central regulator of proteostasis in the cochlea, linking multiple pathological pathways in sensorineural hearing loss and proposing UPS-targeted interventions for treatment.
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Key points
- The ubiquitin-proteasome system (UPS) is essential for protein degradation and proteostasis in the cochlea.
- UPS dysfunction is implicated in the pathogenesis of multiple sensorineural hearing loss types.
- The UPS acts as a central hub linking various cellular pathways involved in hearing loss, such as mitophagy, oxidative stress, and inflammation.
- Targeting UPS components offers potential therapeutic strategies for restoring cochlear proteostasis and treating hearing loss.
AI-generated from the title and abstract; the full text is not read.
Abstract
Abstract The ubiquitin–proteasome system (UPS) is the major machinery for intracellular protein degradation, playing an indispensable role in maintaining proteostasis across all tissues. Sensorineural hearing loss affects billions of people worldwide and is characterized by the irreversible degeneration of cochlear hair cells and spiral ganglion neurons. However, effective treatments remain unavailable. Although UPS dysfunction has been increasingly recognized in aging and neurodegenerative diseases, its specific contributions to cochlear development, homeostasis, and hearing disorders have received relatively limited attention. This review systematically summarizes the roles of UPS-mediated proteostasis in the auditory system. Major UPS related components including E1/E2/E3 ubiquitin ligases, deubiquitinating enzymes and proteasome subunits were discussed in this review. A comprehensive summary of the importance of UPS in cochlear development and the maintenance of inner ear auditory function has been made. Various UPS molecules and their potential pathogenic mechanisms or related signaling pathways are summarized in different types of hearing loss, including congenital hearing loss, age-related hearing loss, noise-induced hearing loss, and drug-related hearing loss. This review first highlights the tissue-specific functional divergence of UPS among cochlear structures. It also proposes the UPS as a central regulatory hub linking multiple pathological pathways in hearing loss including mitophagy, autophagy, oxidative stress, DNA damage repair, endoplasmic reticulum (ER) associated degradation, and NF-κB mediated inflammation. Finally, the emerging therapeutic strategies that target UPS components to restore cochlear proteostasis and hearing loss are discussed. These insights lay a solid foundation for developing UPS-targeted interventions for sensorineural hearing loss, in order to achieve precise treatments according to cochlear cell-specific ubiquitinomics in the future.
The authors' abstract, as published at the source. Molecular Biomedicine, 2026 · DOI ↗
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Field: Sensory Systems
Sensory SystemsNeuroscience