Role of Proteasomes in Inflammation

The ubiquitin–proteasome system (UPS) is involved in multiple cellular functions including the regulation of protein homeostasis, major histocompatibility (MHC) class I antigen processing, cell cycle proliferation and signaling. In humans, proteasome loss-of-function mutations result in autoinflamma...

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Published inJournal of clinical medicine Vol. 10; no. 8; p. 1783
Main Authors Goetzke, Carl Christoph, Ebstein, Frédéric, Kallinich, Tilmann
Format Journal Article
LanguageEnglish
Published Switzerland MDPI AG 20.04.2021
MDPI
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ISSN2077-0383
2077-0383
DOI10.3390/jcm10081783

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Summary:The ubiquitin–proteasome system (UPS) is involved in multiple cellular functions including the regulation of protein homeostasis, major histocompatibility (MHC) class I antigen processing, cell cycle proliferation and signaling. In humans, proteasome loss-of-function mutations result in autoinflammation dominated by a prominent type I interferon (IFN) gene signature. These genomic alterations typically cause the development of proteasome-associated autoinflammatory syndromes (PRAAS) by impairing proteasome activity and perturbing protein homeostasis. However, an abnormal increased proteasomal activity can also be found in other human inflammatory diseases. In this review, we cast a light on the different clinical aspects of proteasomal activity in human disease and summarize the currently studied therapeutic approaches.
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ISSN:2077-0383
2077-0383
DOI:10.3390/jcm10081783