AAA+ proteases: the first line of defense against mitochondrial damage

Metabolic control of adult neural stem cell self-renewal by the mitochondrial protease YME1L

(2022) Gulzar A. Wani et al.   Cell Reports

Organismal and Cellular Stress Responses upon Disruption of Mitochondrial Lonp1 Protease

(2022) Eirini Taouktsi et al.   Cells

Mitochondrial quality control: from molecule to organelle

(2021) Alba Roca-Portoles et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

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

(2021) Sylvia Torres-Odio et al.   JOURNAL OF IMMUNOLOGY

Proteomic analysis demonstrates the role of the quality control protease LONP1 in mitochondrial protein aggregation

(2021) Karen Pollecker et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Structures of the human LONP1 protease reveal regulatory steps involved in protease activation

(2021) Mia Shin et al.   Nature Communications

Mitochondrial Lon protease is a gatekeeper for proteins newly imported into the matrix

(2021) Yuichi Matsushima et al.   Communications Biology

Mitochondrial Proteases: Multifaceted Regulators of Mitochondrial Plasticity

(2020) Soni Deshwal et al.   Annual Review of Biochemistry

Mitochondria-Associated Proteostasis

(2020) Linhao Ruan et al.   Annual Review of Biophysics

Mitochondrial ClpX activates an essential biosynthetic enzyme through partial unfolding

(2020) Julia R Kardon et al.   eLife

Structures of the ATP-fueled ClpXP proteolytic machine bound to protein substrate

(2020) Xue Fei et al.   eLife

PINK1 and Parkin mitochondrial quality control: a source of regional vulnerability in Parkinson’s disease

(2020) Preston Ge et al.   Molecular Neurodegeneration

Mitochondrial Quality Control and Cellular Proteostasis: Two Sides of the Same Coin

(2020) Justin M. Quiles et al.   Frontiers in Physiology

Mitochondrial Metabolism as a Target for Cancer Therapy

(2020) Karthik Vasan et al.   Cell Metabolism

Inactivation of the mitochondrial protease Afg3l2 results in severely diminished respiratory chain activity and widespread defects in mitochondrial gene expression

(2020) Gautam Pareek et al.   PLoS Genetics

Mitochondrial proteins: from biogenesis to functional networks

(2019) Nikolaus Pfanner et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Autophagy accounts for approximately one-third of mitochondrial protein turnover and is protein selective

(2019) Evelyn S. Vincow et al.   Autophagy

Alpha-synuclein suppresses mitochondrial protease ClpP to trigger mitochondrial oxidative damage and neurotoxicity

(2019) Di Hu et al.   ACTA NEUROPATHOLOGICA

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

Mitochondrial electron transport chain, ROS generation and uncoupling (Review)

(2019) Ru‑Zhou Zhao et al.   INTERNATIONAL JOURNAL OF MOLECULAR MEDICINE

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

(2019) Daria Picchioni et al.   Cell Reports

TDP-43 induces mitochondrial damage and activates the mitochondrial unfolded protein response

(2019) Peng Wang et al.   PLoS Genetics

Unique Structural Features of the Mitochondrial AAA+ Protease AFG3L2 Reveal the Molecular Basis for Activity in Health and Disease

(2019) Cristina Puchades et al.   MOLECULAR CELL

Mitochondria—Striking a balance between host and endosymbiont

(2019) Richard J. Youle   SCIENCE

Mitochondrial Dynamics and Its Involvement in Disease

(2019) David C. Chan   Annual Review of Pathology-Mechanisms of Disease

Mitochondrial AAA proteases: A stairway to degradation

(2019) Tyler E. Steele et al.   MITOCHONDRION

TDP-43 proteinopathy and mitochondrial abnormalities in neurodegeneration

(2019) Ju Gao et al.   MOLECULAR AND CELLULAR NEUROSCIENCE

Lipid signalling drives proteolytic rewiring of mitochondria by YME1L

(2019) Thomas MacVicar et al.   NATURE

Mitochondria as multifaceted regulators of cell death

(2019) Florian J. Bock et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Expanding the Clinical Spectrum of LONP1-Related Mitochondrial Cytopathy

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

Dissecting Substrate Specificities of the Mitochondrial AFG3L2 Protease

(2018) Bojian Ding et al.   BIOCHEMISTRY

m-AAA proteases, mitochondrial calcium homeostasis and neurodegeneration

(2018) Maria Patron et al.   CELL RESEARCH

Mitophagy and Quality Control Mechanisms in Mitochondrial Maintenance

(2018) Sarah Pickles et al.   CURRENT BIOLOGY

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

Basal mitophagy is widespread inDrosophilabut minimally affected by loss of Pink1 or parkin

(2018) Juliette J. Lee et al.   JOURNAL OF CELL BIOLOGY

Loss of the Drosophila m-AAA mitochondrial protease paraplegin results in mitochondrial dysfunction, shortened lifespan, and neuronal and muscular degeneration

(2018) Gautam Pareek et al.   Cell Death & Disease

Mitochondrial Lon sequesters and stabilizes p53 in the matrix to restrain apoptosis under oxidative stress via its chaperone activity

(2018) Ya-Ju Sung et al.   Cell Death & Disease

The Close Encounter Between Alpha-Synuclein and Mitochondria

(2018) Mattia Vicario et al.   Frontiers in Neuroscience

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

A mitochondrial FUNDC1/HSC70 interaction organizes the proteostatic stress response at the risk of cell morbidity

(2018) Yanjun Li et al.   EMBO JOURNAL

Loss of the mitochondrial i‐AAA protease YME1L leads to ocular dysfunction and spinal axonopathy

(2018) Hans‐Georg Sprenger et al.   EMBO Molecular Medicine

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 novel mutation in the proteolytic domain of LONP1 causes atypical CODAS syndrome

(2017) Takehiko Inui et al.   JOURNAL OF HUMAN GENETICS

Identification of a Degradation Signal Sequence within Substrates of the Mitochondrial i-AAA Protease

(2017) Anthony J. Rampello et al.   JOURNAL OF MOLECULAR BIOLOGY

Cytosolic proteostasis through importing of misfolded proteins into mitochondria

(2017) Linhao Ruan et al.   NATURE

A fluorescence-based imaging method to measure in vitro and in vivo mitophagy using mt-Keima

(2017) Nuo Sun et al.   Nature Protocols

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

Proteolytic control of the mitochondrial calcium uniporter complex

(2017) Chen-Wei Tsai et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Structure of the mitochondrial inner membrane AAA+ protease YME1 gives insight into substrate processing

(2017) Cristina Puchades et al.   SCIENCE

Drosophila protease ClpXP specifically degrades DmLRPPRC1 controlling mitochondrial mRNA and translation

(2017) Yuichi Matsushima et al.   Scientific Reports

PINK1 Primes Parkin-Mediated Ubiquitination of PARIS in Dopaminergic Neuronal Survival

(2017) Yunjong Lee et al.   Cell Reports

Maintenance and Expression of Mammalian Mitochondrial DNA

(2016) Claes M. Gustafsson et al.   Annual Review of Biochemistry

The Transcription Factor ATF5 Mediates a Mammalian Mitochondrial UPR

(2016) Christopher J. Fiorese et al.   CURRENT BIOLOGY

CLPP coordinates mitoribosomal assembly through the regulation of ERAL1 levels

(2016) Karolina Szczepanowska et al.   EMBO JOURNAL

The membrane scaffold SLP2 anchors a proteolytic hub in mitochondria containing PARL and the i ‐AAA protease YME1L

(2016) Timothy Wai et al.   EMBO REPORTS

Loss of CLPP alleviates mitochondrial cardiomyopathy without affecting the mammalian UPRmt

(2016) Dominic Seiferling et al.   EMBO REPORTS

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

mito-QC illuminates mitophagy and mitochondrial architecture in vivo

(2016) Thomas G. McWilliams et al.   JOURNAL OF CELL BIOLOGY

Protection of scaffold protein Isu from degradation by the Lon protease Pim1 as a component of Fe–S cluster biogenesis regulation

(2016) Szymon J. Ciesielski et al.   MOLECULAR BIOLOGY OF THE CELL

The m -AAA Protease Associated with Neurodegeneration Limits MCU Activity in Mitochondria

(2016) Tim König et al.   MOLECULAR CELL

Mitochondrial unfolded protein response controls matrix pre-RNA processing and translation

(2016) Christian Münch et al.   NATURE

Engineered AAA+ proteases reveal principles of proteolysis at the mitochondrial inner membrane

(2016) Hui Shi et al.   Nature Communications

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

(2016) Kenneth Robert Pryde et al.   Cell Reports

Reciprocal Degradation of YME1L and OMA1 Adapts Mitochondrial Proteolytic Activity during Stress

(2016) T. Kelly Rainbolt et al.   Cell Reports

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

(2016) Jae Ho Seo et al.   PLOS BIOLOGY

Homozygous YME1L1 mutation causes mitochondriopathy with optic atrophy and mitochondrial network fragmentation

(2016) Bianca Hartmann 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

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

Loss of Drosophila i-AAA protease, dYME1L, causes abnormal mitochondria and apoptotic degeneration

(2015) Y Qi et al.   CELL DEATH AND DIFFERENTIATION

Evolution of Mitochondria as Signaling Organelles

(2015) Navdeep S. Chandel   Cell Metabolism

The ubiquitin kinase PINK1 recruits autophagy receptors to induce mitophagy

(2015) Michael Lazarou et al.   NATURE

New roles for mitochondrial proteases in health, ageing and disease

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

Survivin promotes oxidative phosphorylation, subcellular mitochondrial repositioning, and tumor cell invasion

(2015) Dayana B. Rivadeneira et al.   Science Signaling

Survivin promotes oxidative phosphorylation, subcellular mitochondrial repositioning, and tumor cell invasion

(2015) Dayana B. Rivadeneira et al.   Science Signaling

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

Mutations in the SPG7 gene cause chronic progressive external ophthalmoplegia through disordered mitochondrial DNA maintenance

(2014) Gerald Pfeffer et al.   BRAIN

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

(2014) Sukru Anil Dogan et al.   Cell Metabolism

Making Proteins in the Powerhouse

(2014) B. Martin Hällberg et al.   Cell Metabolism

Proteolytic Cleavage of Opa1 Stimulates Mitochondrial Inner Membrane Fusion and Couples Fusion to Oxidative Phosphorylation

(2014) Prashant Mishra et al.   Cell Metabolism

Loss of the m-AAA protease subunit AFG3L2 causes mitochondrial transport defects and tau hyperphosphorylation

(2014) A. K. Kondadi et al.   EMBO JOURNAL

YME1L degradation reduces mitochondrial proteolytic capacity during oxidative stress

(2014) T. K. Rainbolt et al.   EMBO REPORTS

Purkinje neuron Ca2+ influx reduction rescues ataxia in SCA28 model

(2014) Francesca Maltecca et al.   JOURNAL OF CLINICAL INVESTIGATION

Parkin and PINK1: much more than mitophagy

(2014) Leslie A. Scarffe et al.   TRENDS IN NEUROSCIENCES

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

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

PINK1-Parkin Pathway Activity Is Regulated by Degradation of PINK1 in the Mitochondrial Matrix

(2014) Ruth E. Thomas et al.   PLoS Genetics

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

Stress-Regulated Translational Attenuation Adapts Mitochondrial Protein Import through Tim17A Degradation

(2013) T. Kelly Rainbolt et al.   Cell Metabolism

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

The PINK1-Parkin pathway promotes both mitophagy and selective respiratory chain turnover in vivo

(2013) E. S. Vincow et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

EMRE Is an Essential Component of the Mitochondrial Calcium Uniporter Complex

(2013) Y. Sancak et al.   SCIENCE

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

(2013) Fabian Fischer et al.   Nature Communications

The mitochondrial permeability transition pore: a mystery solved?

(2013) Paolo Bernardi   Frontiers in Physiology

Sites of reactive oxygen species generation by mitochondria oxidizing different substrates

(2013) Casey L. Quinlan et al.   Redox Biology

Mitochondria: In Sickness and in Health

(2012) Jodi Nunnari et al.   CELL

Mitochondrial quality control: a matter of life and death for neurons

(2012) Elena I Rugarli et al.   EMBO JOURNAL

Mitochondrial processing peptidase regulates PINK1 processing, import and Parkin recruitment

(2012) Andrew W Greene et al.   EMBO REPORTS

AFG3L2 supports mitochondrial protein synthesis and Purkinje cell survival

(2012) Eva R. Almajan et al.   JOURNAL OF CLINICAL INVESTIGATION

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

(2012) Bin Lu et al.   MOLECULAR CELL

Cysteine desulfurase Nfs1 and Pim1 protease control levels of Isu, the Fe-S cluster biogenesis scaffold

(2012) J.-Y. Song et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mitochondrial Import Efficiency of ATFS-1 Regulates Mitochondrial UPR Activation

(2012) A. M. Nargund et al.   SCIENCE

Mitochondrial Fission, Fusion, and Stress

(2012) R. J. Youle et al.   SCIENCE

Mitochondrial Quality Control Mediated by PINK1 and Parkin: Links to Parkinsonism

(2012) D. Narendra et al.   Cold Spring Harbor Perspectives in Biology

Protective Coupling of Mitochondrial Function and Protein Synthesis via the eIF2α Kinase GCN-2

(2012) Brooke M. Baker et al.   PLoS Genetics

AAA+ Proteases: ATP-Fueled Machines of Protein Destruction

(2011) Robert T. Sauer et al.   Annual Review of Biochemistry

Recent Advances in the Genetics of Parkinson's Disease

(2011) Ian Martin et al.   Annual Review of Genomics and Human Genetics

PARIS (ZNF746) Repression of PGC-1α Contributes to Neurodegeneration in Parkinson's Disease

(2011) Joo-Ho Shin et al.   CELL

Integrative genomics identifies MCU as an essential component of the mitochondrial calcium uniporter

(2011) Joshua M. Baughman et al.   NATURE

ER Tubules Mark Sites of Mitochondrial Division

(2011) J. R. Friedman et al.   SCIENCE

Whole-Exome Sequencing Identifies Homozygous AFG3L2 Mutations in a Spastic Ataxia-Neuropathy Syndrome Linked to Mitochondrial m-AAA Proteases

(2011) Tyler Mark Pierson et al.   PLoS Genetics

Regulation of mitochondrial phospholipids by Ups1/PRELI-like proteins depends on proteolysis and Mdm35

(2010) Christoph Potting et al.   EMBO JOURNAL

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

Mitochondrial membrane potential regulates PINK1 import and proteolytic destabilization by PARL

(2010) Seok Min Jin et al.   JOURNAL OF CELL BIOLOGY

Mutations in the mitochondrial protease gene AFG3L2 cause dominant hereditary ataxia SCA28

(2010) Daniela Di Bella et al.   NATURE GENETICS

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

PINK1 Is Selectively Stabilized on Impaired Mitochondria to Activate Parkin

(2010) Derek P. Narendra et al.   PLOS BIOLOGY

Atg32 Is a Mitochondrial Protein that Confers Selectivity during Mitophagy

(2009) Tomotake Kanki et al.   DEVELOPMENTAL CELL

Regulation of OPA1 processing and mitochondrial fusion bym-AAA protease isoenzymes and OMA1

(2009) Sarah Ehses et al.   JOURNAL OF CELL BIOLOGY

Ups1p and Ups2p antagonistically regulate cardiolipin metabolism in mitochondria

(2009) Yasushi Tamura et al.   JOURNAL OF CELL BIOLOGY

Haploinsufficiency of AFG3L2, the Gene Responsible for Spinocerebellar Ataxia Type 28, Causes Mitochondria-Mediated Purkinje Cell Dark Degeneration

(2009) F. Maltecca et al.   JOURNAL OF NEUROSCIENCE

How mitochondria produce reactive oxygen species

(2008) Michael P. Murphy   BIOCHEMICAL JOURNAL

Recognition of misfolded proteins by Lon, a AAA+ protease

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

Parkin is recruited selectively to impaired mitochondria and promotes their autophagy

(2008) Derek Narendra et al.   JOURNAL OF CELL BIOLOGY

The Mitochondrial Protease AFG3L2 Is Essential for Axonal Development

(2008) F. Maltecca et al.   JOURNAL OF NEUROSCIENCE