Proteostasis Perturbations and Their Roles in Causing Sterile Inflammation and Autoinflammatory Diseases

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 perturbati...

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Published in	Cells (Basel, Switzerland) Vol. 11; no. 9; p. 1422
Main Authors	Papendorf, Jonas Johannes, Krüger, Elke, Ebstein, Frédéric
Format	Journal Article
Language	English
Published	Switzerland MDPI AG 22.04.2022 MDPI
Subjects	Amino acids autoinflammation Autophagy Biosynthesis Endoplasmic reticulum Etiology Homeostasis Immune response Immune system Inflammation Inflammatory diseases Innate immunity Kinases Pathogens Phosphorylation proteasomopathies Protein biosynthesis proteinopathies Proteins Proteomes proteostasis Quality control Review ribosomopathies Sensors Surveillance autoinflammation ribosomopathies proteostasis proteasomopathies proteinopathies
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ISSN	2073-4409 2073-4409
DOI	10.3390/cells11091422

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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.
AbstractList	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. 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.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.
Author	Papendorf, Jonas Johannes Ebstein, Frédéric Krüger, Elke
AuthorAffiliation	Institut für Medizinische Biochemie und Molekularbiologie, Universitätsmedizin Greifswald, Ferdinand-Sauerbruch-Straße, 17475 Greifswald, Germany; jonasjohannes.papendorf@med.uni-greifswald.de
AuthorAffiliation_xml	– name: Institut für Medizinische Biochemie und Molekularbiologie, Universitätsmedizin Greifswald, Ferdinand-Sauerbruch-Straße, 17475 Greifswald, Germany; jonasjohannes.papendorf@med.uni-greifswald.de
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BackLink	https://www.ncbi.nlm.nih.gov/pubmed/35563729$$D View this record in MEDLINE/PubMed
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CitedBy_id	crossref_primary_10_1002_cmdc_202400326 crossref_primary_10_3390_cells11172705 crossref_primary_10_3390_cells13211774 crossref_primary_10_1016_j_jbspin_2024_105719 crossref_primary_10_1016_j_gim_2024_101120 crossref_primary_10_1016_j_molmet_2023_101755 crossref_primary_10_1051_medsci_2023221 crossref_primary_10_1038_s41584_024_01184_8 crossref_primary_10_1016_j_celrep_2024_115094 crossref_primary_10_1016_j_ajhg_2024_05_016 crossref_primary_10_1186_s12979_023_00348_6 crossref_primary_10_1016_j_gendis_2023_101130 crossref_primary_10_1111_imr_13299
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Keywords	autoinflammation ribosomopathies proteostasis proteasomopathies proteinopathies
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SubjectTerms	Amino acids autoinflammation Autophagy Biosynthesis Endoplasmic reticulum Etiology Homeostasis Immune response Immune system Inflammation Inflammatory diseases Innate immunity Kinases Pathogens Phosphorylation proteasomopathies Protein biosynthesis proteinopathies Proteins Proteomes proteostasis Quality control Review ribosomopathies Sensors Surveillance
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Title	Proteostasis Perturbations and Their Roles in Causing Sterile Inflammation and Autoinflammatory Diseases
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