Control of mitochondrial integrity in ageing and disease

Mitochondrial mutations and aging: random drift is insufficient to explain the accumulation of mitochondrial deletion mutants in short-lived animals

(2013) Axel Kowald et al.   AGING CELL

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

The Scaffold Protein Atg11 Recruits Fission Machinery to Drive Selective Mitochondria Degradation by Autophagy

(2013) Kai Mao et al.   DEVELOPMENTAL CELL

Persistent damage induces mitochondrial DNA degradation

(2013) Inna N. Shokolenko et al.   DNA REPAIR

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

Mitochondrial DNA Rearrangements in Health and Disease-A Comprehensive Study

(2013) Joana Damas et al.   HUMAN MUTATION

Mitochondrial DNA Ligase Is Dispensable for the Viability of Cultured Cells but Essential for mtDNA Maintenance

(2013) Inna N. Shokolenko et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mitochondrial mutations and ageing: Can mitochondrial deletion mutants accumulate via a size based replication advantage?

(2013) Axel Kowald et al.   JOURNAL OF THEORETICAL BIOLOGY

Late onset motoneuron disorder caused by mitochondrial Hsp60 chaperone deficiency in mice

(2013) Raffaella Magnoni et al.   NEUROBIOLOGY OF DISEASE

Phenotypic heterogeneity of the 8344A>G mtDNA "MERRF" mutation

(2013) M. Mancuso et al.   NEUROLOGY

A Genome-Wide Longitudinal Transcriptome Analysis of the Aging Model Podospora anserine

(2013) Oliver Philipp et al.   PLoS One

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

Age-dependent dissociation of ATP synthase dimers and loss of inner-membrane cristae in mitochondria

(2013) B. Daum et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The Maintenance of Mitochondrial DNA Integrity--Critical Analysis and Update

(2013) M. Alexeyev et al.   Cold Spring Harbor Perspectives in Biology

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

(2013) Fabian Fischer et al.   Nature Communications

Mitochondrial quality control by the ubiquitin–proteasome system: Figure 1

(2012) Eric B. Taylor et al.   BIOCHEMICAL SOCIETY TRANSACTIONS

A role for ubiquitinylation and the cytosolic proteasome in turnover of mitochondrial uncoupling protein 1 (UCP1)

(2012) Kieran J. Clarke et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Unmasking a temperature-dependent effect of theP. anserinai-AAA protease on aging and development

(2012) Andrea Weil et al.   CELL CYCLE

The pathways of mitophagy for quality control and clearance of mitochondria

(2012) G Ashrafi et al.   CELL DEATH AND DIFFERENTIATION

Substrate- and Isoform-Specific Proteome Stability in Normal and Stressed Cardiac Mitochondria

(2012) Edward Lau et al.   CIRCULATION RESEARCH

Oxidants and not alkylating agents induce rapid mtDNA loss and mitochondrial dysfunction

(2012) Amy M. Furda et al.   DNA REPAIR

OXPHOS mutations and neurodegeneration

(2012) Werner J H Koopman et al.   EMBO JOURNAL

Mitochondrial processing peptidase regulates PINK1 processing, import and Parkin recruitment

(2012) Andrew W Greene et al.   EMBO REPORTS

Maintenance of mitochondrial genome distribution by mitochondrial AAA+ protein ClpX

(2012) Katsumi Kasashima et al.   EXPERIMENTAL CELL RESEARCH

Respiratory dysfunction by AFG3L2 deficiency causes decreased mitochondrial calcium uptake via organellar network fragmentation

(2012) F. Maltecca et al.   HUMAN MOLECULAR GENETICS

AFG3L2 supports mitochondrial protein synthesis and Purkinje cell survival

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

YME1L controls the accumulation of respiratory chain subunits and is required for apoptotic resistance, cristae morphogenesis, and cell proliferation

(2012) Lukas Stiburek et al.   MOLECULAR BIOLOGY OF THE CELL

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

(2012) Bin Lu et al.   MOLECULAR CELL

OPA1 mutations induce mtDNA proliferation in leukocytes of patients with dominant optic atrophy

(2012) K. S. Sitarz et al.   NEUROLOGY

Revisiting the issue of mitochondrial DNA content in optic mitochondriopathies

(2012) L. Iommarini et al.   NEUROLOGY

Mitochondrial DNA deletions and depletion within paraspinal muscles

(2012) G. R. Campbell et al.   NEUROPATHOLOGY AND APPLIED NEUROBIOLOGY

Monogenic Mitochondrial Disorders

(2012) Werner J.H. Koopman et al.   NEW ENGLAND JOURNAL OF MEDICINE

Mitochondrial nucleoid interacting proteins support mitochondrial protein synthesis

(2012) J. He et al.   NUCLEIC ACIDS RESEARCH

Biological Roles of the Podospora anserina Mitochondrial Lon Protease and the Importance of Its N-Domain

(2012) Céline Adam et al.   PLoS One

Blood Cell Mitochondrial DNA Content and Premature Ovarian Aging

(2012) Marco Bonomi et al.   PLoS One

Mitochondrial quality control: an integrated network of pathways

(2012) Fabian Fischer et al.   TRENDS IN BIOCHEMICAL SCIENCES

Deceleration of Fusion–Fission Cycles Improves Mitochondrial Quality Control during Aging

(2012) Marc Thilo Figge et al.   PLoS Computational Biology

Comparison of Mitochondrial Mutation Spectra in Ageing Human Colonic Epithelium and Disease: Absence of Evidence for Purifying Selection in Somatic Mitochondrial DNA Point Mutations

(2012) Laura C. Greaves et al.   PLoS Genetics

Loss of Prohibitin Membrane Scaffolds Impairs Mitochondrial Architecture and Leads to Tau Hyperphosphorylation and Neurodegeneration

(2012) Carsten Merkwirth et al.   PLoS Genetics

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

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

Multitasking in the mitochondrion by the ATP-dependent Lon protease

(2011) Sundararajan Venkatesh et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Mitochondrial transcription factor A overexpression and base excision repair deficiency in the inner ear of rats with d-galactose-induced aging

(2011) Yi Zhong et al.   FEBS Journal

Molecular mechanisms and physiologic functions of mitochondrial dynamics

(2011) H. Otera et al.   JOURNAL OF BIOCHEMISTRY

Mitochondrial DNA polymerase   mutations: an ever expanding molecular and clinical spectrum

(2011) S. Tang et al.   JOURNAL OF MEDICAL GENETICS

Parkin promotes the ubiquitination and degradation of the mitochondrial fusion factor mitofusin 1

(2011) Liliane Glauser et al.   JOURNAL OF NEUROCHEMISTRY

Differences in the accumulation of mitochondrial defects with age in mice and humans

(2011) Laura C. Greaves et al.   MECHANISMS OF AGEING AND DEVELOPMENT

Macromolecular organization of ATP synthase and complex I in whole mitochondria

(2011) K. M. Davies et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Quality Control of Mitochondrial Proteostasis

(2011) M. J. Baker et al.   Cold Spring Harbor Perspectives in Biology

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

Cyclophilin D links programmed cell death and organismal aging in Podospora anserina

(2010) Diana Brust et al.   AGING CELL

Functional Null Mutations of MSRB3 Encoding Methionine Sulfoxide Reductase Are Associated with Human Deafness DFNB74

(2010) Zubair M. Ahmed et al.   AMERICAN JOURNAL OF HUMAN GENETICS

The investigation and diagnosis of pathogenic mitochondrial DNA mutations in human urothelial cells

(2010) John K. Blackwood et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

DNA repair in organelles: Pathways, organization, regulation, relevance in disease and aging

(2010) Pierre Boesch et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Aging: ROS or TOR

(2010) Mikhail V Blagosklonny   CELL CYCLE

Inhibition of Drp1-dependent mitochondrial fragmentation and apoptosis by a polypeptide antagonist of calcineurin

(2010) G M Cereghetti et al.   CELL DEATH AND DIFFERENTIATION

Inactivation of the hereditary spastic paraplegia-associated Hspd1 gene encoding the Hsp60 chaperone results in early embryonic lethality in mice

(2010) Jane H. Christensen et al.   CELL STRESS & CHAPERONES

Mitochondrial DNA replication and disease: insights from DNA polymerase γ mutations

(2010) Jeffrey D. Stumpf et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Deletion of PaAif2 and PaAmid2, two genes encoding mitochondrial AIF-like oxidoreductases of Podospora anserina, leads to increased stress tolerance and lifespan extension

(2010) Diana Brust et al.   CURRENT GENETICS

Defects in multiple complexes of the respiratory chain are present in ageing human colonic crypts

(2010) Laura C. Greaves et al.   EXPERIMENTAL GERONTOLOGY

Impaired quality control of mitochondria: Aging from a new perspective

(2010) Tobias A. Weber et al.   EXPERIMENTAL GERONTOLOGY

Mitofusin 1 and mitofusin 2 are ubiquitinated in a PINK1/parkin-dependent manner upon induction of mitophagy

(2010) Matthew E. Gegg et al.   HUMAN MOLECULAR GENETICS

Oxygen-induced changes in mitochondrial DNA and DNA repair enzymes in aging rat lens

(2010) Yi Zhang et al.   MECHANISMS OF AGEING AND DEVELOPMENT

The Matrix Peptide Exporter HAF-1 Signals a Mitochondrial UPR by Activating the Transcription Factor ZC376.7 in C. elegans

(2010) Cole M. Haynes et al.   MOLECULAR CELL

PINK1/Parkin-mediated mitophagy is dependent on VDAC1 and p62/SQSTM1

(2010) Sven Geisler et al.   NATURE CELL BIOLOGY

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

(2010) Daniela Di Bella et al.   NATURE GENETICS

Mitochondrial DNA Mutations Induce Mitochondrial Dysfunction, Apoptosis and Sarcopenia in Skeletal Muscle of Mitochondrial DNA Mutator Mice

(2010) Asimina Hiona et al.   PLoS One

Analysis of proteome dynamics in the mouse brain

(2010) J. C. Price et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

Mammalian Mitochondrial Complex I: Biogenesis, Regulation, and Reactive Oxygen Species Generation

(2009) Werner J.H. Koopman et al.   ANTIOXIDANTS & REDOX SIGNALING

A New Mitochondrial Transfer RNAPro Gene Mutation Associated With Myoclonic Epilepsy With Ragged-Red Fibers and Other Neurological Features

(2009) Emma L. Blakely et al.   ARCHIVES OF NEUROLOGY

Role of mitofusin 2 mutations in the physiopathology of Charcot–Marie–Tooth disease type 2A

(2009) Romain Cartoni et al.   EXPERIMENTAL NEUROLOGY

Overexpression of Tfam protects mitochondria against β-amyloid-induced oxidative damage in SH-SY5Y cells

(2009) Shangcheng Xu et al.   FEBS Journal

Mechanisms of cross-talk between the ubiquitin-proteasome and autophagy-lysosome systems

(2009) Viktor I. Korolchuk et al.   FEBS LETTERS

Mitochondrial dynamics-fusion, fission, movement, and mitophagy-in neurodegenerative diseases

(2009) H. Chen et al.   HUMAN MOLECULAR GENETICS

Reversal of the Mitochondrial Phenotype and Slow Development of Oxidative Biomarkers of Aging in Long-livedMclk1+/−Mice

(2009) Jérôme Lapointe et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

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

Batteries not included: diagnosis and management of mitochondrial disease

(2009) R. McFarland et al.   JOURNAL OF INTERNAL MEDICINE

Age-related decline in stress responses of human myocardium may not be explained by changes in mtDNA

(2009) Francis Miller et al.   MECHANISMS OF AGEING AND DEVELOPMENT

Region-specific changes in mitochondrial D-loop in aged rat CNS

(2009) Simone C. McInerny et al.   MECHANISMS OF AGEING AND DEVELOPMENT

Autocatalytic Processing of m-AAA Protease Subunits in Mitochondria

(2009) Mirko Koppen et al.   MOLECULAR BIOLOGY OF THE CELL

Increasing organismal healthspan by enhancing mitochondrial protein quality control

(2009) Karin Luce et al.   NATURE CELL BIOLOGY

Mitochondrial fission factor Drp1 is essential for embryonic development and synapse formation in mice

(2009) Naotada Ishihara et al.   NATURE CELL BIOLOGY

Neuromuscular disease presentation with three genetic defects involving two genomes

(2009) Mazhor Al-Dosary et al.   NEUROMUSCULAR DISORDERS

Oxidative stress induces degradation of mitochondrial DNA

(2009) Inna Shokolenko et al.   NUCLEIC ACIDS RESEARCH

Age-Dependent Down-Regulation of Mitochondrial 8-Oxoguanine DNA Glycosylase in SAM-P/8 Mouse Brain and Its Effect on Brain Aging

(2009) Feng Tian et al.   REJUVENATION RESEARCH

Prohibitin and mitochondrial biology

(2009) Marta Artal-Sanz et al.   TRENDS IN ENDOCRINOLOGY AND METABOLISM

Mitochondrial Hsp60 Chaperonopathy Causes an Autosomal-Recessive Neurodegenerative Disorder Linked to Brain Hypomyelination and Leukodystrophy

(2008) Daniella Magen et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Mitochondrial protein quality control: Implications in ageing

(2008) Bertrand Friguet et al.   Biotechnology Journal

The Mitochondrial Proteome: From Inventory to Function

(2008) Chris Meisinger et al.   CELL

A Mitochondrial Protein Compendium Elucidates Complex I Disease Biology

(2008) David J. Pagliarini et al.   CELL

Age-related decline in mitochondrial DNA copy number in isolated human pancreatic islets

(2008) L. M. Cree et al.   DIABETOLOGIA

Quality control of mitochondria: protection against neurodegeneration and ageing

(2008) Takashi Tatsuta et al.   EMBO JOURNAL

Fission and selective fusion govern mitochondrial segregation and elimination by autophagy

(2008) Gilad Twig et al.   EMBO JOURNAL

Human prohibitin 1 maintains the organization and stability of the mitochondrial nucleoids

(2008) Katsumi Kasashima et al.   EXPERIMENTAL CELL RESEARCH

Prohibitins control cell proliferation and apoptosis by regulating OPA1-dependent cristae morphogenesis in mitochondria

(2008) C. Merkwirth et al.   GENES & DEVELOPMENT

A clinical, genetic, and biochemical characterization ofSPG7mutations in a large cohort of patients with hereditary spastic paraplegia

(2008) Alessia Arnoldi et al.   HUMAN MUTATION

Overexpression of Mitochondrial Methionine Sulfoxide Reductase B2 Protects Leukemia Cells from Oxidative Stress-induced Cell Death and Protein Damage

(2008) Filipe Cabreiro et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Parkin is recruited selectively to impaired mitochondria and promotes their autophagy

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

Age-related activation of mitochondrial caspase-independent apoptotic signaling in rat gastrocnemius muscle

(2008) Emanuele Marzetti et al.   MECHANISMS OF AGEING AND DEVELOPMENT

Fetal–juvenile origins of point mutations in the adult human tracheal–bronchial epithelium: Absence of detectable effects of age, gender or smoking status

(2008) Hiroko Sudo et al.   MUTATION RESEARCH-FUNDAMENTAL AND MOLECULAR MECHANISMS OF MUTAGENESIS

Essential role for Nix in autophagic maturation of erythroid cells

(2008) Hector Sandoval et al.   NATURE

Mature ribosomes are selectively degraded upon starvation by an autophagy pathway requiring the Ubp3p/Bre5p ubiquitin protease

(2008) Claudine Kraft et al.   NATURE CELL BIOLOGY

A reduction of mitochondrial DNA molecules during embryogenesis explains the rapid segregation of genotypes

(2008) Lynsey M Cree et al.   NATURE GENETICS

Decreased expression of the mitochondrial matrix proteases Lon and ClpP in cells from a patient with hereditary spastic paraplegia (SPG13)

(2008) J. Hansen et al.   NEUROSCIENCE

Apoptosis pathways in fungal growth, development and ageing

(2008) Andrea Hamann et al.   TRENDS IN MICROBIOLOGY

Strong Purifying Selection in Transmission of Mammalian Mitochondrial DNA

(2008) James Bruce Stewart et al.   PLOS BIOLOGY

OPA1 mutations induce mitochondrial DNA instability and optic atrophy 'plus' phenotypes

(2007) P. Amati-Bonneau et al.   BRAIN