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Loss of Mitochondrial Protease CLPP Activates Type I IFN Responses through the Mitochondrial DNA–cGAS–STING Signaling Axis
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What this work says
Outgoing claims: the papers this work cites, builds on, or argues with.
inferredcites →https://doi.org/10.1002/j.1460-2075.1988.tb03259.xopenalex-snapshot
Male sterility of transgenic mice carrying exogenous mouse interferon‐beta gene under the control of the metallothionein enhancer‐promoter.
inferredcites →https://doi.org/10.1006/viro.2000.0736openalex-snapshot
Vesicular Stomatitis Virus Glycoprotein Containing the Entire Green Fluorescent Protein on Its Cytoplasmic Domain Is Incorporated Efficiently into Virus Particles
inferredcites →https://doi.org/10.1007/978-981-10-2513-6_1openalex-snapshot
Physiological and Pathological Functions of Mitochondrial Proteases
inferredcites →https://doi.org/10.1007/s10048-020-00609-2openalex-snapshot
Loss of mitochondrial ClpP, Lonp1, and Tfam triggers transcriptional induction of Rnf213, a susceptibility factor for moyamoya disease
inferredcites →https://doi.org/10.1007/s11481-016-9683-8openalex-snapshot
Mitochondrial Quality Control Proteases in Neuronal Welfare
inferredcites →https://doi.org/10.1016/j.ajhg.2013.02.013openalex-snapshot
Perrault Syndrome Is Caused by Recessive Mutations in CLPP, Encoding a Mitochondrial ATP-Dependent Chambered Protease
inferredcites →https://doi.org/10.1016/j.bbamcr.2011.06.007openalex-snapshot
ClpXP, an ATP-powered unfolding and protein-degradation machine
inferredcites →https://doi.org/10.1016/j.ccell.2019.03.014openalex-snapshot
Mitochondrial ClpP-Mediated Proteolysis Induces Selective Cancer Cell Lethality
inferredcites →https://doi.org/10.1016/j.cell.2014.11.037openalex-snapshot
Apoptotic Caspases Prevent the Induction of Type I Interferons by Mitochondrial DNA
inferredcites →https://doi.org/10.1016/j.devcel.2007.07.016openalex-snapshot
ClpP Mediates Activation of a Mitochondrial Unfolded Protein Response in C. elegans
inferredcites →https://doi.org/10.1016/j.freeradbiomed.2015.12.021openalex-snapshot
Down-regulation of the mitochondrial matrix peptidase ClpP in muscle cells causes mitochondrial dysfunction and decreases cell proliferation
inferredcites →https://doi.org/10.1016/j.gde.2016.03.005openalex-snapshot
Human mitochondrial DNA replication machinery and disease
inferredcites →https://doi.org/10.1016/j.it.2016.12.004openalex-snapshot
Crosstalk between Cytoplasmic RIG-I and STING Sensing Pathways
inferredcites →https://doi.org/10.1016/j.mito.2017.11.004openalex-snapshot
UPR mt coordinates immunity to maintain mitochondrial homeostasis and animal fitness
inferredcites →https://doi.org/10.1016/j.tox.2017.07.016openalex-snapshot
Mitochondrial dysfunction as a trigger of innate immune responses and inflammation
inferredcites →https://doi.org/10.1016/j.yexcr.2010.10.008openalex-snapshot
Human mitochondrial transcription factor A is required for the segregation of mitochondrial DNA in cultured cells
inferredcites →https://doi.org/10.1016/j.yexcr.2012.07.012openalex-snapshot
Maintenance of mitochondrial genome distribution by mitochondrial AAA+ protein ClpX
inferredcites →https://doi.org/10.1016/s0021-9258(18)37906-7openalex-snapshot
Interferon expression in the testes of transgenic mice leads to sterility.
inferredcites →https://doi.org/10.1021/acschembio.8b00124openalex-snapshot
The Role of ClpP Protease in Bacterial Pathogenesis and Human Diseases
inferredcites →https://doi.org/10.1021/acschembio.9b00222openalex-snapshot
Mitochondrial Protease ClpP is a Target for the Anticancer Compounds ONC201 and Related Analogues
inferredcites →https://doi.org/10.1038/cr.2018.16openalex-snapshot
UPRmt regulation and output: a stress response mediated by mitochondrial-nuclear communication
inferredcites →https://doi.org/10.1038/nature09907openalex-snapshot
A diverse range of gene products are effectors of the type I interferon antiviral response
inferredcites →https://doi.org/10.1038/nature13818openalex-snapshot
Mitochondrial UPR-regulated innate immunity provides resistance to pathogen infection
inferredcites →https://doi.org/10.1038/nature14156openalex-snapshot
Mitochondrial DNA stress primes the antiviral innate immune response
inferredcites →https://doi.org/10.1038/nbt.1511openalex-snapshot
MaxQuant enables high peptide identification rates, individualized p.p.b.-range mass accuracies and proteome-wide protein quantification
inferredcites →https://doi.org/10.1038/nri.2017.21openalex-snapshot
Mitochondrial DNA in innate immune responses and inflammatory pathology
inferredcites →https://doi.org/10.1038/nrm3984openalex-snapshot
New roles for mitochondrial proteases in health, ageing and disease
inferredcites →https://doi.org/10.1038/s12276-018-0196-9openalex-snapshot
Unique roles of tryptophanyl-tRNA synthetase in immune control and its therapeutic implications
inferredcites →https://doi.org/10.1038/s12276-019-0333-0openalex-snapshot
STING signaling and host defense against microbial infection
inferredcites →https://doi.org/10.1038/s41586-018-0363-0openalex-snapshot
Mitochondrial double-stranded RNA triggers antiviral signalling in humans
inferredcites →https://doi.org/10.1038/s41598-018-30311-1openalex-snapshot
Perrault syndrome type 3 caused by diverse molecular defects in CLPP
inferredcites →https://doi.org/10.1038/sj.embor.7400878openalex-snapshot
Herpes simplex virus eliminates host mitochondrial DNA
inferredcites →https://doi.org/10.1073/pnas.1301951110openalex-snapshot
Dynamics of nucleoid structure regulated by mitochondrial fission contributes to cristae reformation and release of cytochrome c
inferredcites →https://doi.org/10.1073/pnas.1611113114openalex-snapshot
Chronic innate immune activation of TBK1 suppresses mTORC1 activity and dysregulates cellular metabolism
inferredcites →https://doi.org/10.1073/pnas.1716937115openalex-snapshot
STING-dependent translation inhibition restricts RNA virus replication
inferredcites →https://doi.org/10.1080/01485010490474823openalex-snapshot
INTERFERON α-2B MAY IMPAIR TESTICULAR HISTOLOGY INCLUDING SPERMATOGENESIS IN A RAT MODEL
inferredcites →https://doi.org/10.1084/jem.20151464openalex-snapshot
RNase H2 catalytic core Aicardi-Goutières syndrome–related mutant invokes cGAS–STING innate immune-sensing pathway in mice
inferredcites →https://doi.org/10.1089/ars.2015.6407openalex-snapshot
The Roles of Mitochondrial Damage-Associated Molecular Patterns in Diseases
inferredcites →https://doi.org/10.1093/hmg/ddp281openalex-snapshot
Inhibition of mitochondrial fission favours mutant over wild-type mitochondrial DNA
inferredcites →https://doi.org/10.1093/hmg/ddt338openalex-snapshot
Loss of mitochondrial peptidase Clpp leads to infertility, hearing loss plus growth retardation via accumulation of CLPX, mtDNA and inflammatory factors
inferredcites →https://doi.org/10.1111/acel.12784openalex-snapshot
Mitochondrial unfolded protein response gene Clpp is required to maintain ovarian follicular reserve during aging, for oocyte competence, and development of pre‐implantation embryos
inferredcites →https://doi.org/10.1111/j.1572-0241.2004.30372.xopenalex-snapshot
Sudden Hearing Loss in Patients with Chronic Hepatitis C Treated with Pegylated Interferon/Ribavirin
inferredcites →https://doi.org/10.1126/science.aav4011openalex-snapshot
VDAC oligomers form mitochondrial pores to release mtDNA fragments and promote lupus-like disease
inferredcites →https://doi.org/10.1126/science.aaw9855openalex-snapshot
Mitochondria—Striking a balance between host and endosymbiont
inferredcites →https://doi.org/10.1128/jvi.02087-08openalex-snapshot
Herpes Simplex Virus UL12.5 Targets Mitochondria through a Mitochondrial Localization Sequence Proximal to the N Terminus
inferredcites →https://doi.org/10.1128/jvi.72.9.7563-7568.1998openalex-snapshot
Incorporation of the Green Fluorescent Protein into the Herpes Simplex Virus Type 1 Capsid
inferredcites →https://doi.org/10.1155/2017/8060949openalex-snapshot
Mitochondrial Nucleoid: Shield and Switch of the Mitochondrial Genome
inferredcites →https://doi.org/10.15252/embj.201694253openalex-snapshot
CLPP coordinates mitoribosomal assembly through the regulation of ERAL1 levels
inferredcites →https://doi.org/10.15252/embr.201745009openalex-snapshot
Loss of mitochondrial protease ClpP protects mice from diet‐induced obesity and insulin resistance
inferredcites →https://doi.org/10.15252/embr.201745126openalex-snapshot
CLPP deficiency protects against metabolic syndrome but hinders adaptive thermogenesis
inferredcites →https://doi.org/10.3389/fncel.2019.00046openalex-snapshot
Herpes Simplex Virus Type 1 Infection of the Central Nervous System: Insights Into Proposed Interrelationships With Neurodegenerative Disorders
inferredcites →https://doi.org/10.3389/fneur.2016.00203openalex-snapshot
Specific MRI Abnormalities Reveal Severe Perrault Syndrome due to CLPP Defects
inferredcites →https://doi.org/10.3390/ijms20184523openalex-snapshot
Global Proteome of LonP1+/− Mouse Embryonal Fibroblasts Reveals Impact on Respiratory Chain, but No Interdependence between Eral1 and Mitoribosomes
inferredcites →https://doi.org/10.4103/1012-5574.168359openalex-snapshot
Study of the effect of combined interferon and ribavirin therapy on the hearing profile of hepatitis C virus patients
inferredcites →https://doi.org/10.5772/intechopen.73036openalex-snapshot
Modulation of Mitochondria During Viral Infections
inferredcites →https://doi.org/10.7554/elife.47491openalex-snapshot
Tight nuclear tethering of cGAS is essential for preventing autoreactivity
What points at this work
Incoming claims: where this work appears in other papers’ reference lists and arguments.
inferred← citeshttps://doi.org/10.2139/ssrn.4663953openalex-extracted
An Unconventional Mechanism of IL-1β Secretion that Requires Type I IFN in SLE Monocytes
inferred← citeshttps://doi.org/10.1002/ana.27081openalex-snapshot
Interferon Stimulated Gene Expression Is a Biomarker for Primary Mitochondrial Disease
inferred← citeshttps://doi.org/10.1002/cpz1.372openalex-snapshot
Assessing Mitochondrial DNA Release into the Cytosol and Subsequent Activation of Innate Immune‐related Pathways in Mammalian Cells
inferred← citeshttps://doi.org/10.1002/mco2.70827openalex-snapshot
Cellular Senescence and Aging: Mechanisms, Disease Convergence, and Therapeutic Frontiers
inferred← citeshttps://doi.org/10.1002/med.21929openalex-snapshot
Mitochondrial quality control proteases and their modulation for cancer therapy
inferred← citeshttps://doi.org/10.1007/s00430-022-00742-9openalex-snapshot
Mitochondria: intracellular sentinels of infections
inferred← citeshttps://doi.org/10.1007/s10048-021-00657-2openalex-snapshot
Increased presence of nuclear DNAJA3 and upregulation of cytosolic STAT1 and of nucleic acid sensors trigger innate immunity in the ClpP-null mouse
inferred← citeshttps://doi.org/10.1007/s10495-025-02217-7openalex-snapshot
Targeting VDAC1 to protect against mitochondria-linked cell death pathways: apoptosis, pyroptosis, ferroptosis, and associated diseases
inferred← citeshttps://doi.org/10.1016/j.arr.2023.101955openalex-snapshot
Mitochondrial dysfunction in aging
inferred← citeshttps://doi.org/10.1016/j.arr.2026.103174openalex-snapshot
Immunotherapy for senescent cell clearance: Hallmarks, strategies and translational challenges
inferred← citeshttps://doi.org/10.1016/j.bbamem.2024.184329openalex-snapshot
Effect of VBIT-4 on the functional activity of isolated mitochondria and cell viability
inferred← citeshttps://doi.org/10.1016/j.biochi.2024.06.005openalex-snapshot
Mitochondrial proteases modulate mitochondrial stress signalling and cellular homeostasis in health and disease
inferred← citeshttps://doi.org/10.1016/j.cca.2026.120832openalex-snapshot
A novel CLPP variant in a Pakistani family with Perrault syndrome associated with recurrent fevers
inferred← citeshttps://doi.org/10.1016/j.celrep.2022.111774openalex-snapshot
Mitochondrial cristae architecture protects against mtDNA release and inflammation
inferred← citeshttps://doi.org/10.1016/j.cophys.2022.100571openalex-snapshot
Mitochondrial function and dysfunction in innate immunity
inferred← citeshttps://doi.org/10.1016/j.envpol.2025.127007openalex-snapshot
ACR-16/α7-nicotinic receptor signaling mediates nicotine-induced mitochondrial dysfunction and suppression of host defense against gram-negative bacteria
inferred← citeshttps://doi.org/10.1016/j.ijbiomac.2025.142368openalex-snapshot
Immp2l gene knockout induces granulosa cell senescence by activation of cGAS-STING pathway via TFAM-mediated mtDNA leakage
inferred← citeshttps://doi.org/10.1016/j.jbc.2025.110430openalex-snapshot
The ability of SAMHD1-deficient monocytes to trigger the Type I IFN response depends on cGAS and mitochondrial DNA
inferred← citeshttps://doi.org/10.1016/j.mito.2024.101929openalex-snapshot
The role and mechanism of VDAC1 in type 2 diabetes: An underestimated target of environmental pollutants
inferred← citeshttps://doi.org/10.1016/j.molcel.2026.01.008openalex-snapshot
Mitochondria as sources and targets of cellular signaling
inferred← citeshttps://doi.org/10.1016/j.redox.2026.104412openalex-snapshot
CLPP loss confers sex-specific protection from renal ischemia reperfusion injury
inferred← citeshttps://doi.org/10.1016/j.xcrm.2024.101840openalex-snapshot
Chemical activation of mitochondrial ClpP to modulate energy metabolism of CD4+ T cell for inflammatory bowel diseases treatment
inferred← citeshttps://doi.org/10.1016/j.ymgme.2026.110278openalex-snapshot
Hyperinflammatory syndromes in primary mitochondrial disease: A three-center case series and focused review
inferred← citeshttps://doi.org/10.1038/s41467-025-59907-8openalex-snapshot
Caspase-11 drives macrophage hyperinflammation in models of Polg-related mitochondrial disease
inferred← citeshttps://doi.org/10.1038/s41574-021-00626-7openalex-snapshot
Mitochondrial and metabolic dysfunction in ageing and age-related diseases
inferred← citeshttps://doi.org/10.1038/s42003-024-07143-zopenalex-snapshot
Targeting protein homeostasis with small molecules as a strategy for the development of pan-coronavirus antiviral therapies
inferred← citeshttps://doi.org/10.1038/s42255-025-01332-8openalex-snapshot
2-hydroxyglutarate mediates whitening of brown adipocytes coupled to nuclear softening upon mitochondrial dysfunction
inferred← citeshttps://doi.org/10.1042/bst20221525openalex-snapshot
Mitochondrial DNA in cell death and inflammation
inferred← citeshttps://doi.org/10.1083/jcb.202310005openalex-snapshot
Regulation of proteostasis and innate immunity via mitochondria-nuclear communication
inferred← citeshttps://doi.org/10.1093/brain/awag037openalex-snapshot
Mitochondrial DNA release and inflammation in mitochondrial disease pathogenesis
inferred← citeshttps://doi.org/10.1093/hmg/ddad062openalex-snapshot
Activation of the cGAS-STING innate immune response in cells with deficient mitochondrial topoisomerase TOP1MT
inferred← citeshttps://doi.org/10.1093/hmg/ddae031openalex-snapshot
Mitochondrial DNA release and sensing in innate immune responses
inferred← citeshttps://doi.org/10.1096/fj.202600927ropenalex-snapshot
The Genetic and Epigenetic Mechanisms of mtDNA in Regulation of Inflammation and Metabolism
inferred← citeshttps://doi.org/10.1101/2022.05.13.491896openalex-snapshot
Borrelia burgdorferi engages mammalian type I interferon responses via the cGAS-STING pathway
inferred← citeshttps://doi.org/10.1101/2023.08.03.551696openalex-snapshot
An unconventional mechanism of IL-1β secretion that requires Type I IFN in lupus monocytes
inferred← citeshttps://doi.org/10.1101/2025.03.13.642980openalex-snapshot
Chronic lysosome damage boosts interferon responses to Palbociclib dependent upon the mitochondrion
inferred← citeshttps://doi.org/10.1111/all.70157openalex-snapshot
Large‐Scale Blood Proteomic Analysis Across Different Inflammatory Skin Conditions Reveals Extensive Immune Dysregulation With Distinct Biomarker Profiles
inferred← citeshttps://doi.org/10.1136/jitc-2024-011377openalex-snapshot
Acasunlimab, an Fc-inert PD-L1×4-1BB bispecific antibody, combined with PD-1 blockade potentiates antitumor immunity via complementary immune modulatory effects
inferred← citeshttps://doi.org/10.1146/annurev-biochem-032620-104401openalex-snapshot
Mitochondrial DNA Release in Innate Immune Signaling
inferred← citeshttps://doi.org/10.1182/blood.2024026340openalex-snapshot
The mitochondrial protease ClpP is a metabolic vulnerability and an immunogenic trigger against multiple myeloma
inferred← citeshttps://doi.org/10.1186/s12964-025-02042-0openalex-snapshot
Mitochondrial DNA signals driving immune responses: Why, How, Where?
inferred← citeshttps://doi.org/10.1186/s12974-025-03363-0openalex-snapshot
Mitochondrial DNA leakage: underlying mechanisms and therapeutic implications in neurological disorders
inferred← citeshttps://doi.org/10.1242/dmm.052804openalex-snapshot
Advances in diagnosis of mitochondrial dysfunction and primary mitochondrial disease
inferred← citeshttps://doi.org/10.20944/preprints202105.0021.v1openalex-snapshot
Prominent Increases of Nuclear DNAJA3and Cytosolic STAT1 with Nucleic Acid Sensors Underlie Innate Immunity Activation in ClpP-null Mouse
inferred← citeshttps://doi.org/10.20944/preprints202204.0245.v1openalex-snapshot
ClpP-Deletion Causes Azoospermia, with Meiosis-I Delay and Insufficient Biosynthesis of Spermatid Factors, Due to Mitochondrial Dysfunction with Accumulation of Perrault Proteins ERAL1, PEO1, and HARS2
inferred← citeshttps://doi.org/10.20944/preprints202205.0033.v1openalex-snapshot
Clpp-Deletion Causes Azoospermia, with Meiosis-I Delay and Insufficient Biosynthesis of Spermatid Factors, Due to Mitochondrial Dysfunction with Accumulation of Perrault Proteins ERAL1, PEO1, and HARS2
inferred← citeshttps://doi.org/10.3389/fcell.2021.720490openalex-snapshot
Metabolism and Innate Immunity Meet at the Mitochondria
inferred← citeshttps://doi.org/10.3389/fcell.2022.796066openalex-snapshot
Role of Mitochondrial Nucleic Acid Sensing Pathways in Health and Patho-Physiology
inferred← citeshttps://doi.org/10.3389/fimmu.2021.729763openalex-snapshot
Mitochondrial Nucleic Acid as a Driver of Pathogenic Type I Interferon Induction in Mendelian Disease
inferred← citeshttps://doi.org/10.3389/fimmu.2022.941579openalex-snapshot
African Swine Fever Virus Structural Protein p17 Inhibits cGAS-STING Signaling Pathway Through Interacting With STING
inferred← citeshttps://doi.org/10.3389/fimmu.2026.1761658openalex-snapshot
The role of mitochondrial proteases in inflammation and immunity
inferred← citeshttps://doi.org/10.3389/fimmu.2026.1935135openalex-snapshot
Sterile inflammation in LHON: mitochondrial stress, innate immune signaling, and implications for AAV gene therapy
inferred← citeshttps://doi.org/10.3390/biom14020241openalex-snapshot
CLPP-Null Eukaryotes with Excess Heme Biosynthesis Show Reduced L-arginine Levels, Probably via CLPX-Mediated OAT Activation
inferred← citeshttps://doi.org/10.3390/cells10123354openalex-snapshot
Inactivity of Peptidase ClpP Causes Primary Accumulation of Mitochondrial Disaggregase ClpX with Its Interacting Nucleoid Proteins, and of mtDNA
inferred← citeshttps://doi.org/10.3390/cells11152370openalex-snapshot
The Bacterial ClpXP-ClpB Family Is Enriched with RNA-Binding Protein Complexes
inferred← citeshttps://doi.org/10.3390/cells12010052openalex-snapshot
CLPP Depletion Causes Diplotene Arrest; Underlying Testis Mitochondrial Dysfunction Occurs with Accumulation of Perrault Proteins ERAL1, PEO1, and HARS2
inferred← citeshttps://doi.org/10.3390/genes15060694openalex-snapshot
Knockout Mouse Studies Show That Mitochondrial CLPP Peptidase and CLPX Unfoldase Act in Matrix Condensates near IMM, as Fast Stress Response in Protein Assemblies for Transcript Processing, Translation, and Heme Production
inferred← citeshttps://doi.org/10.3390/ijms22168523openalex-snapshot
Mitochondria-Induced Immune Response as a Trigger for Neurodegeneration: A Pathogen from Within
inferred← citeshttps://doi.org/10.4049/jimmunol.2200354openalex-snapshot
Borrelia burgdorferi Engages Mammalian Type I IFN Responses via the cGAS–STING Pathway
inferred← citeshttps://doi.org/10.7287/peerj.14350v0.1/reviews/2openalex-snapshot
Peer Review #2 of "AAA+ proteases: the first line of defense against mitochondrial damage (v0.1)"
inferred← citeshttps://doi.org/10.7717/peerj.14350openalex-snapshot
AAA+ proteases: the first line of defense against mitochondrial damage
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