Loss of Mitochondrial Protease CLPP Activates Type I IFN Responses through the Mitochondrial DNA–cGAS–STING Signaling Axis

VDAC oligomers form mitochondrial pores to release mtDNA fragments and promote lupus-like disease

(2020) Jeonghan Kim et al.   SCIENCE

Loss of mitochondrial ClpP, Lonp1, and Tfam triggers transcriptional induction of Rnf213, a susceptibility factor for moyamoya disease

(2020) Jana Key et al.   NEUROGENETICS

Herpes Simplex Virus Type 1 Infection of the Central Nervous System: Insights Into Proposed Interrelationships With Neurodegenerative Disorders

(2019) Luisa F. Duarte et al.   Frontiers in Cellular Neuroscience

Mitochondrial Protease ClpP is a Target for the Anticancer Compounds ONC201 and Related Analogues

(2019) Paul R. Graves et al.   ACS Chemical Biology

Mitochondrial ClpP-Mediated Proteolysis Induces Selective Cancer Cell Lethality

(2019) Jo Ishizawa et al.   CANCER CELL

Global Proteome of LonP1+/− Mouse Embryonal Fibroblasts Reveals Impact on Respiratory Chain, but No Interdependence between Eral1 and Mitoribosomes

(2019) Jana Key et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

Mitochondria—Striking a balance between host and endosymbiont

(2019) Richard J. Youle   SCIENCE

Tight nuclear tethering of cGAS is essential for preventing autoreactivity

(2019) Hannah E Volkman et al.   eLife

The Role of ClpP Protease in Bacterial Pathogenesis and Human Diseases

(2018) Vaibhav Bhandari et al.   ACS Chemical Biology

UPRmt regulation and output: a stress response mediated by mitochondrial-nuclear communication

(2018) Andrew Melber et al.   CELL RESEARCH

Loss of mitochondrial protease ClpP protects mice from diet‐induced obesity and insulin resistance

(2018) Shylesh Bhaskaran et al.   EMBO REPORTS

CLPP deficiency protects against metabolic syndrome but hinders adaptive thermogenesis

(2018) Christina Becker et al.   EMBO REPORTS

UPR mt coordinates immunity to maintain mitochondrial homeostasis and animal fitness

(2018) Siyuan Wang et al.   MITOCHONDRION

STING-dependent translation inhibition restricts RNA virus replication

(2018) Kate M. Franz et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mitochondrial double-stranded RNA triggers antiviral signalling in humans

(2018) Ashish Dhir et al.   NATURE

Perrault syndrome type 3 caused by diverse molecular defects in CLPP

(2018) Erica J. Brodie et al.   Scientific Reports

Mitochondrial Nucleoid: Shield and Switch of the Mitochondrial Genome

(2017) Sung Ryul Lee et al.   Oxidative Medicine and Cellular Longevity

Chronic innate immune activation of TBK1 suppresses mTORC1 activity and dysregulates cellular metabolism

(2017) Maroof Hasan et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Crosstalk between Cytoplasmic RIG-I and STING Sensing Pathways

(2017) Alessandra Zevini et al.   TRENDS IN IMMUNOLOGY

Human mitochondrial DNA replication machinery and disease

(2016) Matthew J Young et al.   CURRENT OPINION IN GENETICS & DEVELOPMENT

CLPP coordinates mitoribosomal assembly through the regulation of ERAL1 levels

(2016) Karolina Szczepanowska et al.   EMBO JOURNAL

Down-regulation of the mitochondrial matrix peptidase ClpP in muscle cells causes mitochondrial dysfunction and decreases cell proliferation

(2016) Sathyaseelan S. Deepa et al.   FREE RADICAL BIOLOGY AND MEDICINE

RNase H2 catalytic core Aicardi-Goutières syndrome–related mutant invokes cGAS–STING innate immune-sensing pathway in mice

(2016) Vladislav Pokatayev et al.   JOURNAL OF EXPERIMENTAL MEDICINE

Mitochondrial Quality Control Proteases in Neuronal Welfare

(2016) Roman M. Levytskyy et al.   Journal of Neuroimmune Pharmacology

Specific MRI Abnormalities Reveal Severe Perrault Syndrome due to CLPP Defects

(2016) Tom E. J. Theunissen et al.   Frontiers in Neurology

The Roles of Mitochondrial Damage-Associated Molecular Patterns in Diseases

(2015) Kiichi Nakahira et al.   ANTIOXIDANTS & REDOX SIGNALING

Mitochondrial DNA stress primes the antiviral innate immune response

(2015) A. Phillip West et al.   NATURE

New roles for mitochondrial proteases in health, ageing and disease

(2015) Pedro M. Quirós et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Apoptotic Caspases Prevent the Induction of Type I Interferons by Mitochondrial DNA

(2014) Anthony Rongvaux et al.   CELL

Mitochondrial UPR-regulated innate immunity provides resistance to pathogen infection

(2014) Mark W. Pellegrino et al.   NATURE

Perrault Syndrome Is Caused by Recessive Mutations in CLPP, Encoding a Mitochondrial ATP-Dependent Chambered Protease

(2013) Emma M. Jenkinson et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Loss of mitochondrial peptidase Clpp leads to infertility, hearing loss plus growth retardation via accumulation of CLPX, mtDNA and inflammatory factors

(2013) S. Gispert et al.   HUMAN MOLECULAR GENETICS

Dynamics of nucleoid structure regulated by mitochondrial fission contributes to cristae reformation and release of cytochrome c

(2013) R. Ban-Ishihara et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Maintenance of mitochondrial genome distribution by mitochondrial AAA+ protein ClpX

(2012) Katsumi Kasashima et al.   EXPERIMENTAL CELL RESEARCH

ClpXP, an ATP-powered unfolding and protein-degradation machine

(2011) Tania A. Baker et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

A diverse range of gene products are effectors of the type I interferon antiviral response

(2011) John W. Schoggins et al.   NATURE

Human mitochondrial transcription factor A is required for the segregation of mitochondrial DNA in cultured cells

(2010) Katsumi Kasashima et al.   EXPERIMENTAL CELL RESEARCH

Inhibition of mitochondrial fission favours mutant over wild-type mitochondrial DNA

(2009) Adriana Malena et al.   HUMAN MOLECULAR GENETICS

Herpes Simplex Virus UL12.5 Targets Mitochondria through a Mitochondrial Localization Sequence Proximal to the N Terminus

(2009) J. A. Corcoran et al.   JOURNAL OF VIROLOGY

MaxQuant enables high peptide identification rates, individualized p.p.b.-range mass accuracies and proteome-wide protein quantification

(2008) Jürgen Cox et al.   NATURE BIOTECHNOLOGY