{"database":"biostudies-literature","file_versions":[],"scores":null,"additional":{"submitter":["Torres-Odio S"],"funding":["NHLBI NIH HHS","Office of the Assistant Secretary of Defense for Health Affairs, U.S. Department of Defense","National Heart, Lung, and Blood Institute, National Institutes of Health"],"pagination":["1890-1900"],"full_dataset_link":["https://www.ebi.ac.uk/biostudies/studies/S-EPMC8026707"],"repository":["biostudies-literature"],"omics_type":["Unknown"],"volume":["206(8)"],"pubmed_abstract":["Caseinolytic mitochondrial matrix peptidase proteolytic subunit (CLPP) is a serine protease that degrades damaged or misfolded mitochondrial proteins. CLPP-null mice exhibit growth retardation, deafness, and sterility, resembling human Perrault syndrome, but also display immune system alterations. However, the molecular mechanisms and signaling pathways underlying immunological changes in CLPP-null mice remain unclear. In this study, we report the steady-state activation of type I IFN signaling and antiviral gene expression in CLPP-deficient cells and tissues, resulting in marked resistance to RNA and DNA virus infection. Depletion of the cyclic GMP-AMP (cGAS)-stimulator of IFN genes (STING) DNA sensing pathway reduces steady-state IFN-I signaling and abrogates the broad antiviral phenotyp"],"journal":["Journal of immunology (Baltimore, Md. : 1950)"],"pubmed_title":["Loss of Mitochondrial Protease CLPP Activates Type I IFN Responses through the Mitochondrial DNA-cGAS-STING Signaling Axis."],"pmcid":["PMC8026707"],"funding_grant_id":["R01HL148153","W81XWH-20-1-0150","W81XWH-17-1-0052","R01 HL148153"],"pubmed_authors":["West AP","Torres-Odio S","Wittig I","Menissy SS","Lei Y","Key J","Auburger G","Gispert S","Maletzko A"],"additional_accession":[]},"is_claimable":false,"name":"Loss of Mitochondrial Protease CLPP Activates Type I IFN Responses through the Mitochondrial DNA-cGAS-STING Signaling Axis.","description":"Caseinolytic mitochondrial matrix peptidase proteolytic subunit (CLPP) is a serine protease that degrades damaged or misfolded mitochondrial proteins. CLPP-null mice exhibit growth retardation, deafness, and sterility, resembling human Perrault syndrome, but also display immune system alterations. However, the molecular mechanisms and signaling pathways underlying immunological changes in CLPP-null mice remain unclear. In this study, we report the steady-state activation of type I IFN signaling and antiviral gene expression in CLPP-deficient cells and tissues, resulting in marked resistance to RNA and DNA virus infection. Depletion of the cyclic GMP-AMP (cGAS)-stimulator of IFN genes (STING) DNA sensing pathway reduces steady-state IFN-I signaling and abrogates the broad antiviral phenotyp","dates":{"release":"2021-01-01T00:00:00Z","publication":"2021 Apr","modification":"2026-05-09T20:09:57.839Z","creation":"2025-02-19T00:56:26.682Z"},"accession":"S-EPMC8026707","cross_references":{"pubmed":["33731338"],"doi":["10.4049/jimmunol.2001016"]}}