ReviewSeminars in immunopathology2015
Dysfunction in protein clearance by the proteasome: impact on autoinflammatory diseases.
Review in Seminars in immunopathology, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 44 papers.
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Who cites it
44 citing papers in PubMed, 71 citations in OpenAlex.
- Article
- Molecular mechanisms in rare proteasomopathies.EULAR rheumatology open · 2026Review
- Expression and DNA methylation of 20S proteasome subunits as prognostic and resistance markers in cancer.Molecular oncology · 2025Article
- Article
- Knockdown of Sec16 causes early lethality and defective deposition of the protein Rp30 in the eggshell of the vectorFrontiers in cell and developmental biology · 2024Article
- Ectopic USP15 expression inhibits HIV-1 transcription involving changes in YY1 deubiquitination and stability.Frontiers in cellular and infection microbiology · 2024Article
- PSMC3 proteasome subunit variants are associated with neurodevelopmental delay and type I interferon production.Science translational medicine · 2023Article
- Identification of eight novel proteasome variants in five unrelated cases of proteasome-associated autoinflammatory syndromes (PRAAS).Frontiers in immunology · 2023Article
- Immunogenic cell death triggered by impaired deubiquitination in multiple myeloma relies on dysregulated type I interferon signaling.Frontiers in immunology · 2023Article
- Therapeutic Antiaging Strategies.Biomedicines · 2022Review
- Disorders of ubiquitylation: unchained inflammation.Nature reviews. Rheumatology · 2022Review
- Review
- Proteostasis Perturbations and Their Roles in Causing Sterile Inflammation and Autoinflammatory Diseases.Cells · 2022Review
- Chaperone-mediated assembly of the proteasome core particle - recent developments and structural insights.Journal of cell science · 2022Review
- Ingestion of hemozoin by peripheral blood mononuclear cells alters temporal gene expression of ubiquitination processes.Biochemistry and biophysics reports · 2022Article
- Targeting Proteasomes in Cancer and Infectious Disease: A Parallel Strategy to Treat Malignancies and Microbes.Frontiers in cellular and infection microbiology · 2022Review
- Comprehensive Analysis Reveals USP45 as a Novel Putative Oncogene in Pan-Cancer.Frontiers in molecular biosciences · 2022Article
- Review
- Role of Proteasomes in Inflammation.Journal of clinical medicine · 2021Review
- Immune Dysfunction in Mendelian Disorders of POLA1 Deficiency.Journal of clinical immunology · 2021Review
Corrections and comments
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
During innate immune responses, proteostasis is greatly impacted by synthesis of pathogen proteins as well as by inflammatory tissue damage through radicals or other damaging molecules released by phagocytes. An adequate adaptation of cellular clearance pathways to the increased burden of damaged proteins is thus of fundamental importance for cells and tissues to prevent protein aggregation, inclusion body formation, and ultimately cell death. We here review the current understanding of the pivotal role of the ubiquitin proteasome system (UPS) in this proteostasis network. The proteolytic capacity of the UPS can be adjusted by differential gene expression, the incorporation and maturation kinetics of alternative active sites, and the attachment of different regulators. Dysregulation of this fine-tuning is likely to induce cell death but seen more often to promote inflammation as well. The link between proteostasis impairment and inflammation may play a crucial role in autoinflammation as well as in age-related diseases and currently uncharacterized diseases. Recent studies on proteasome-associated autoinflammatory syndromes (PRAAS) discovered that IFN signaling drives the inflammation caused by reduction of degradation capacity. Elucidation of these syndromes will reveal further insights in the understanding of inadequate immune responses. Knowledge related to the diversity of this degradation system will raise the awareness of potential pitfalls in the molecular diagnostics of autoinflammatory syndromes and may help to identify novel drug targets.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.