Emerging perspectives in proteostasis: bridging mechanisms and therapeutics for human diseases

Cellular protein homeostasis, or proteostasis, underpins the integrity, adaptability, and survival of all cells by balancing protein synthesis, folding, trafficking, and degradation. This multilayered network is sustained by coordinated actions of molecular chaperones, the ubiquitin‒proteasome system, autophagy-lysosomal pathways, and organelle-specific quality control programs. When this equilibrium collapses, misfolded, aggregated, or damaged proteins accumulate, driving organelle dysfunction, maladaptive stress signaling, and disease progression. Disruption of proteostasis is now recognized as a unifying pathological hallmark linking neurodegenerative disorders, cancer, cardiovascular and metabolic diseases, and autoimmune conditions. This is particularly consequential in post-mitotic organs such as the heart and brain, which possess limited regenerative capacity and are exceptionally vulnerable to proteotoxic stress. Rapid advances now reveal proteostasis as a multicomponent, cross-compartmental, and dynamically adaptable system, rather than isolated pathways. We frame this complexity through the concept of proteostasis resilience, defined as the ability of cells and tissues to maintain proteome stability under stress, and use it to unify disease mechanisms with therapeutic opportunity. This review integrates mechanistic insights with translational advances, outlining how dysregulation of chaperones, autophagy-mitophagy, the ubiquitin‒proteasome system, and ER stress pathways drive human diseases, while highlighting emerging therapeutic platforms, from pharmacological chaperones and autophagy modulators to targeted protein degradation technologies, CRISPR screens, spatial biology, and AI-guided drug discovery. Together, this systems-level perspective positions proteostasis resilience as a foundational paradigm for understanding disease vulnerability and designing precision proteostasis-based therapies.

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Publication Details

Journal
Signal Transduction and Targeted Therapy
Published
2026-06-08
DOI
https://doi.org/10.1038/s41392-026-02714-4
Primary Topic
Autophagy in Disease and Therapy
Type
article
Field-Weighted Citation Impact
0.00

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article

Emerging perspectives in proteostasis: bridging mechanisms and therapeutics for human diseases

Ashraf Yusuf Rangrez, Ankush Borlepawar, Parvana Hajieva, Hannah Bühringer et al.
Signal Transduction and Targeted Therapy
Autophagy in Disease and Therapy
article

Emerging perspectives in proteostasis: bridging mechanisms and therapeutics for human diseases

Ashraf Yusuf Rangrez, Ankush Borlepawar, Parvana Hajieva, Hannah Bühringer, Marco Neu, Ziqi Ma, Norbert Frey, Anushka Deshpande
article en

Abstract

Cellular protein homeostasis, or proteostasis, underpins the integrity, adaptability, and survival of all cells by balancing protein synthesis, folding, trafficking, and degradation. This multilayered network is sustained by coordinated actions of molecular chaperones, the ubiquitin‒proteasome system, autophagy-lysosomal pathways, and organelle-specific quality control programs. When this equilibrium collapses, misfolded, aggregated, or damaged proteins accumulate, driving organelle dysfunction, maladaptive stress signaling, and disease progression. Disruption of proteostasis is now recognized as a unifying pathological hallmark linking neurodegenerative disorders, cancer, cardiovascular and metabolic diseases, and autoimmune conditions. This is particularly consequential in post-mitotic organs such as the heart and brain, which possess limited regenerative capacity and are exceptionally vulnerable to proteotoxic stress. Rapid advances now reveal proteostasis as a multicomponent, cross-compartmental, and dynamically adaptable system, rather than isolated pathways. We frame this complexity through the concept of proteostasis resilience, defined as the ability of cells and tissues to maintain proteome stability under stress, and use it to unify disease mechanisms with therapeutic opportunity. This review integrates mechanistic insights with translational advances, outlining how dysregulation of chaperones, autophagy-mitophagy, the ubiquitin‒proteasome system, and ER stress pathways drive human diseases, while highlighting emerging therapeutic platforms, from pharmacological chaperones and autophagy modulators to targeted protein degradation technologies, CRISPR screens, spatial biology, and AI-guided drug discovery. Together, this systems-level perspective positions proteostasis resilience as a foundational paradigm for understanding disease vulnerability and designing precision proteostasis-based therapies.

Signal Transduction and Targeted TherapyVol. 11(1)
Heidelberg University (DE), University Hospital Heidelberg (DE), MSH Medical School Hamburg – University of Applied Sciences and Medical University (DE), BioEnergetics (United States) (US), German Centre for Cardiovascular Research (DE), DKFZ-ZMBH Alliance (DE)
Deutsche Forschungsgemeinschaft
Openalex Percentile: Top 6%
Autophagy in Disease and Therapy
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