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- Title
Proteostasis Perturbations and Their Roles in Causing Sterile Inflammation and Autoinflammatory Diseases.
- Authors
Papendorf, Jonas Johannes; Krüger, Elke; Ebstein, Frédéric
- Abstract
Proteostasis, a portmanteau of the words protein and homeostasis, refers to the ability of eukaryotic cells to maintain a stable proteome by acting on protein synthesis, quality control and/or degradation. Over the last two decades, an increasing number of disorders caused by proteostasis perturbations have been identified. Depending on their molecular etiology, such diseases may be classified into ribosomopathies, proteinopathies and proteasomopathies. Strikingly, most—if not all—of these syndromes exhibit an autoinflammatory component, implying a direct cause-and-effect relationship between proteostasis disruption and the initiation of innate immune responses. In this review, we provide a comprehensive overview of the molecular pathogenesis of these disorders and summarize current knowledge of the various mechanisms by which impaired proteostasis promotes autoinflammation. We particularly focus our discussion on the notion of how cells sense and integrate proteostasis perturbations as danger signals in the context of autoinflammatory diseases to provide insights into the complex and multiple facets of sterile inflammation.
- Subjects
AUTOINFLAMMATORY diseases; INFLAMMATION; EUKARYOTIC cells; PROTEIN synthesis; IMMUNE response
- Publication
Cells (2073-4409), 2022, Vol 11, Issue 9, pN.PAG
- ISSN
2073-4409
- Publication type
Article
- DOI
10.3390/cells11091422