Mitochondrial matrix proteases: quality control and beyond

Mitochondrial proteases in human diseases

(2021) Maria Gomez‐Fabra Gala et al.   FEBS LETTERS

Tune instead of destroy: How proteolysis keeps OXPHOS in shape

(2021) Karolina Szczepanowska et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

LONP1 de novo dominant mutation causes mitochondrial encephalopathy with loss of LONP1 chaperone activity and excessive LONP1 proteolytic activity

(2020) Arnaud Besse et al.   MITOCHONDRION

Mitochondrial ClpX activates an essential biosynthetic enzyme through partial unfolding

(2020) Julia R Kardon et al.   eLife

The Mitochondrial Lon Protease: Novel Functions off the Beaten Track?

(2020) Wolfgang Voos et al.   Biomolecules

Mitochondria Targeting as an Effective Strategy for Cancer Therapy

(2020) Poorva Ghosh et al.   INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES

The mouse heart mitochondria N terminome provides insights into ClpXP-mediated proteolysis

(2020) Eduard Hofsetz et al.   MOLECULAR & CELLULAR PROTEOMICS

A salvage pathway maintains highly functional respiratory complex I

(2020) Karolina Szczepanowska et al.   Nature Communications

ClpP regulates breast cancer cell proliferation, invasion and apoptosis by modulating the Src/PI3K/Akt signaling pathway

(2020) Juan Luo et al.   PeerJ

Mitochondrial CLPP2 assists coordination and homeostasis of respiratory complexes

(2020) Jakob Petereit et al.   PLANT PHYSIOLOGY

Quality control of the mitochondrial proteome

(2020) Jiyao Song et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Mitochondrial ClpP serine protease-biological function and emerging target for cancer therapy

(2020) Kazem Nouri et al.   Cell Death & Disease

The proteasome: friend and foe of mitochondrial biogenesis

(2020) Lena Krämer et al.   FEBS LETTERS

Mitochondrial LonP1 protects cardiomyocytes from ischemia/reperfusion injury in vivo

(2019) Sundararajan Venkatesh et al.   JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY

N-degron and C-degron pathways of protein degradation

(2019) Alexander Varshavsky   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mitochondrial ClpP-Mediated Proteolysis Induces Selective Cancer Cell Lethality

(2019) Jo Ishizawa et al.   CANCER CELL

Mitochondrial Protein Synthesis and mtDNA Levels Coordinated through an Aminoacyl-tRNA Synthetase Subunit

(2019) Daria Picchioni et al.   Cell Reports

Mrx6 regulates mitochondrial DNA copy number in S. cerevisiae by engaging the evolutionarily conserved Lon protease Pim1

(2019) Aylin Göke et al.   MOLECULAR BIOLOGY OF THE CELL

Expanding the Clinical Spectrum of LONP1-Related Mitochondrial Cytopathy

(2019) Fady Hannah-Shmouni et al.   Frontiers in Neurology

Regulated Proteolysis in Bacteria

(2018) Samar A. Mahmoud et al.   Annual Review of Biochemistry

Mitochondrial Aconitase in Neurodegenerative Disorders: Role of a Metabolism- related Molecule in Neurodegeneration

(2018) Fariba Khodagholi et al.   CURRENT DRUG TARGETS

CLPP deficiency protects against metabolic syndrome but hinders adaptive thermogenesis

(2018) Christina Becker et al.   EMBO REPORTS

Defective mitochondrial protease LonP1 can cause classical mitochondrial disease

(2018) Bradley Peter et al.   HUMAN MOLECULAR GENETICS

LONP1 Is Required for Maturation of a Subset of Mitochondrial Proteins, and Its Loss Elicits an Integrated Stress Response

(2018) Olga Zurita Rendón et al.   MOLECULAR AND CELLULAR BIOLOGY

Bi-allelic mutations of LONP1 encoding the mitochondrial LonP1 protease cause pyruvate dehydrogenase deficiency and profound neurodegeneration with progressive cerebellar atrophy

(2018) Graeme A M Nimmo et al.   HUMAN MOLECULAR GENETICS

A homozygous missense mutation in ERAL1, encoding a mitochondrial rRNA chaperone, causes Perrault syndrome

(2017) Iliana A. Chatzispyrou et al.   HUMAN MOLECULAR GENETICS

Multi-omics analysis identifies ATF4 as a key regulator of the mitochondrial stress response in mammals

(2017) Pedro M. Quirós et al.   JOURNAL OF CELL BIOLOGY

Conformational control of the bacterial Clp protease by natural product antibiotics

(2017) I. T. Malik et al.   NATURAL PRODUCT REPORTS

Targeting mitochondrial oxidative phosphorylation eradicates therapy-resistant chronic myeloid leukemia stem cells

(2017) Elodie M Kuntz et al.   NATURE MEDICINE

Mutation in humanCLPXelevates levels ofδ-aminolevulinate synthase and protoporphyrin IX to promote erythropoietic protoporphyria

(2017) Yvette Y. Yien et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

A pathology atlas of the human cancer transcriptome

(2017) Mathias Uhlen et al.   SCIENCE

LRPPRC-mediated folding of the mitochondrial transcriptome

(2017) Stefan J. Siira et al.   Nature Communications

Drosophila protease ClpXP specifically degrades DmLRPPRC1 controlling mitochondrial mRNA and translation

(2017) Yuichi Matsushima et al.   Scientific Reports

Transcriptomic and proteomic landscape of mitochondrial dysfunction reveals secondary coenzyme Q deficiency in mammals

(2017) Inge Kühl et al.   eLife

The role of Lon-mediated proteolysis in the dynamics of mitochondrial nucleic acid-protein complexes

(2017) Nina Kunová et al.   Scientific Reports

The peroxisomal Lon protease LonP2 in aging and disease: functions and comparisons with mitochondrial Lon protease LonP1

(2016) Laura C. D. Pomatto et al.   BIOLOGICAL REVIEWS

Disease-Causing SDHAF1 Mutations Impair Transfer of Fe-S Clusters to SDHB

(2016) Nunziata Maio et al.   Cell Metabolism

CLPP coordinates mitoribosomal assembly through the regulation of ERAL1 levels

(2016) Karolina Szczepanowska et al.   EMBO JOURNAL

Loss of CLPP alleviates mitochondrial cardiomyopathy without affecting the mammalian UPRmt

(2016) Dominic Seiferling et al.   EMBO REPORTS

The integrated stress response

(2016) Karolina Pakos‐Zebrucka et al.   EMBO REPORTS

Novel Mechanisms for Heme-dependent Degradation of ALAS1 Protein as a Component of Negative Feedback Regulation of Heme Biosynthesis

(2016) Yoshiko Kubota et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Regulation of Pyruvate Dehydrogenase Kinase 4 in the Heart through Degradation by the Lon Protease in Response to Mitochondrial Substrate Availability

(2016) Clair Crewe et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mitochondrial Lon is over-expressed in high-grade gliomas, and mediates hypoxic adaptation: potential role of Lon as a therapeutic target in glioma

(2016) Kaijun Di et al.   Oncotarget

Discovery and clinical introduction of first-in-class imipridone ONC201

(2016) Joshua E. Allen et al.   Oncotarget

The N-terminal domain plays a crucial role in the structure of a full-length human mitochondrial Lon protease

(2016) Sami Kereïche et al.   Scientific Reports

A LON-ClpP Proteolytic Axis Degrades Complex I to Extinguish ROS Production in Depolarized Mitochondria

(2016) Kenneth Robert Pryde et al.   Cell Reports

The Mitochondrial Unfoldase-Peptidase Complex ClpXP Controls Bioenergetics Stress and Metastasis

(2016) Jae Ho Seo et al.   PLOS BIOLOGY

Mitochondrial dysfunction remodels one-carbon metabolism in human cells

(2016) Xiaoyan Robert Bao et al.   eLife

CODAS Syndrome Is Associated with Mutations of LONP1, Encoding Mitochondrial AAA+ Lon Protease

(2015) Kevin A. Strauss et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Mutations inLONP1, a mitochondrial matrix protease, cause CODAS syndrome

(2015) Esra Dikoglu et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART A

Inhibition of the Mitochondrial Protease ClpP as a Therapeutic Strategy for Human Acute Myeloid Leukemia

(2015) Alicia Cole et al.   CANCER CELL

Mitochondrial ClpX Activates a Key Enzyme for Heme Biosynthesis and Erythropoiesis

(2015) Julia R. Kardon et al.   CELL

Mitochondrial Lon protease at the crossroads of oxidative stress, ageing and cancer

(2015) Marcello Pinti et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Spatial and temporal dynamics of the cardiac mitochondrial proteome

(2015) Edward Lau et al.   Expert Review of Proteomics

Respiratory chain protein turnover rates in mice are highly heterogeneous but strikingly conserved across tissues, ages, and treatments

(2015) Pabalu P. Karunadharma et al.   FASEB JOURNAL

Mitochondrial protein turnover: Methods to measure turnover rates on a large scale

(2015) X’avia C.Y. Chan et al.   JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY

New roles for mitochondrial proteases in health, ageing and disease

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

LON is the master protease that protects against protein aggregation in human mitochondria through direct degradation of misfolded proteins

(2015) Ayenachew Bezawork-Geleta et al.   Scientific Reports

Adaptation of leukemia cells to hypoxic condition through switching the energy metabolism or avoiding the oxidative stress

(2014) Mineaki Goto et al.   BMC CANCER

Tissue-Specific Loss of DARS2 Activates Stress Responses Independently of Respiratory Chain Deficiency in the Heart

(2014) Sukru Anil Dogan et al.   Cell Metabolism

Oxidative Post-Translational Modifications Develop LONP1 Dysfunction in Pressure Overload Heart Failure

(2014) Atsushi Hoshino et al.   Circulation-Heart Failure

Dynamics of the ClpP serine protease: A model for self-compartmentalized proteases

(2014) Kaiyin Liu et al.   CRITICAL REVIEWS IN BIOCHEMISTRY AND MOLECULAR BIOLOGY

Silencing of mitochondrial Lon protease deeply impairs mitochondrial proteome and function in colon cancer cells

(2014) Lara Gibellini et al.   FASEB JOURNAL

Mitochondrial matrix proteostasis is linked to hereditary paraganglioma: LON-mediated turnover of the human flavinylation factor SDH5 is regulated by its interaction with SDHA

(2014) Ayenachew Bezawork-Geleta et al.   FASEB JOURNAL

Mitochondrial cholesterol: mechanisms of import and effects on mitochondrial function

(2014) Laura A. Martin et al.   JOURNAL OF BIOENERGETICS AND BIOMEMBRANES

Oncogene ablation-resistant pancreatic cancer cells depend on mitochondrial function

(2014) Andrea Viale et al.   NATURE

Self-consumption: the interplay of autophagy and apoptosis

(2014) Guillermo Mariño et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

ATP-Dependent Lon Protease Controls Tumor Bioenergetics by Reprogramming Mitochondrial Activity

(2014) Pedro M. Quirós et al.   Cell Reports

Structural basis for DNA-mediated allosteric regulation facilitated by the AAA+module of Lon protease

(2014) Alan Yueh-Luen Lee et al.   ACTA CRYSTALLOGRAPHICA SECTION D-BIOLOGICAL CRYSTALLOGRAPHY

Variants of mitochondrial autophagy: Types 1 and 2 mitophagy and micromitophagy (Type 3)

(2014) John J. Lemasters   Redox Biology

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

Mitonuclear protein imbalance as a conserved longevity mechanism

(2013) Riekelt H. Houtkooper et al.   NATURE

ER-stress-induced transcriptional regulation increases protein synthesis leading to cell death

(2013) Jaeseok Han et al.   NATURE CELL BIOLOGY

Down-Regulating Overexpressed Human Lon in Cervical Cancer Suppresses Cell Proliferation and Bioenergetics

(2013) Xiaobo Nie et al.   PLoS One

Overexpression of Lon contributes to survival and aggressive phenotype of cancer cells through mitochondrial complex I-mediated generation of reactive oxygen species

(2013) C-W Cheng et al.   Cell Death & Disease

Human CLPP reverts the longevity phenotype of a fungal ClpP deletion strain

(2013) Fabian Fischer et al.   Nature Communications

The mitochondrial ATP-dependent Lon protease: a novel target in lymphoma death mediated by the synthetic triterpenoid CDDO and its derivatives

(2012) S. H. Bernstein et al.   BLOOD

Maintenance of mitochondrial genome distribution by mitochondrial AAA+ protein ClpX

(2012) Katsumi Kasashima et al.   EXPERIMENTAL CELL RESEARCH

Diphenylarsinic Acid Promotes Degradation of Glutaminase C by Mitochondrial Lon Protease

(2012) Kayoko Kita et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Substrate recognition and processing by a Walker B mutant of the human mitochondrial AAA+ protein CLPX

(2012) Bradley R. Lowth et al.   JOURNAL OF STRUCTURAL BIOLOGY

Phosphorylation of Human TFAM in Mitochondria Impairs DNA Binding and Promotes Degradation by the AAA+ Lon Protease

(2012) Bin Lu et al.   MOLECULAR CELL

Mitochondrial Import Efficiency of ATFS-1 Regulates Mitochondrial UPR Activation

(2012) A. M. Nargund et al.   SCIENCE

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

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

Inhibition of Mitochondrial Translation as a Therapeutic Strategy for Human Acute Myeloid Leukemia

(2011) Marko Škrtić et al.   CANCER CELL

Lon Peptidase 1 (LONP1)-dependent Breakdown of Mitochondrial 5-Aminolevulinic Acid Synthase Protein by Heme in Human Liver Cells

(2011) Qing Tian et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mitochondrial enzymes are protected from stress-induced aggregation by mitochondrial chaperones and the Pim1/LON protease

(2011) Tom Bender et al.   MOLECULAR BIOLOGY OF THE CELL

Super-resolution microscopy reveals that mammalian mitochondrial nucleoids have a uniform size and frequently contain a single copy of mtDNA

(2011) C. Kukat et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mitochondrial proteases and cancer

(2010) Anne-Laure Bulteau et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Obtusilactone A and (−)-sesamin induce apoptosis in human lung cancer cells by inhibiting mitochondrial Lon protease and activating DNA damage checkpoints

(2010) Hui-Min Wang et al.   CANCER SCIENCE

Crystal structure of Lon protease: molecular architecture of gated entry to a sequestered degradation chamber

(2010) Sun-Shin Cha et al.   EMBO JOURNAL

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

(2010) Katsumi Kasashima et al.   EXPERIMENTAL CELL RESEARCH

Identification of Novel Oxidized Protein Substrates and Physiological Partners of the Mitochondrial ATP-dependent Lon-like Protease Pim1

(2010) Aurélien Bayot et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The mitochondrial UPR - protecting organelle protein homeostasis

(2010) C. M. Haynes et al.   JOURNAL OF CELL SCIENCE

Suborganelle Sensing of Mitochondrial cAMP-Dependent Protein Kinase Activity

(2010) Richard S. Agnes et al.   JOURNAL OF THE AMERICAN CHEMICAL SOCIETY

Functional characterization of the promoter of the human Lon protease gene

(2010) Marcello Pinti et al.   MITOCHONDRION

Mitochondrial Lon protease regulates mitochondrial DNA copy number and transcription by selective degradation of mitochondrial transcription factor A (TFAM)

(2010) Y. Matsushima et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Cyclic AMP Produced inside Mitochondria Regulates Oxidative Phosphorylation

(2009) Rebeca Acin-Perez et al.   Cell Metabolism

Quality control of mitochondria: protection against neurodegeneration and ageing

(2008) Takashi Tatsuta et al.   EMBO JOURNAL

Recognition of misfolded proteins by Lon, a AAA+ protease

(2008) E. Gur et al.   GENES & DEVELOPMENT