Distinct mitochondrial defects trigger the integrated stress response depending on the metabolic state of the cell

Mitochondrial stress is relayed to the cytosol by an OMA1–DELE1–HRI pathway

(2020) Xiaoyan Guo et al.   NATURE

A pathway coordinated by DELE1 relays mitochondrial stress to the cytosol

(2020) Evelyn Fessler et al.   NATURE

Mitochondria, OxPhos, and neurodegeneration: cells are not just running out of gas

(2019) Estela Area-Gomez et al.   JOURNAL OF CLINICAL INVESTIGATION

Activation of GCN2 by the ribosomal P-stalk

(2019) Alison J. Inglis et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Biomarkers and clinical rating scales for sodium pyruvate therapy in patients with mitochondrial disease

(2019) Yasutoshi Koga et al.   MITOCHONDRION

A Compendium of Genetic Modifiers of Mitochondrial Dysfunction Reveals Intra-organelle Buffering

(2019) Tsz-Leung To et al.   CELL

Leigh Syndrome Mouse Model Can Be Rescued by Interventions that Normalize Brain Hyperoxia, but Not HIF Activation

(2019) Isha H. Jain et al.   Cell Metabolism

The ribosomal P-stalk couples amino acid starvation to GCN2 activation in mammalian cells

(2019) Heather P Harding et al.   eLife

NAD(H) and NADP(H) Redox Couples and Cellular Energy Metabolism

(2018) Wusheng Xiao et al.   ANTIOXIDANTS & REDOX SIGNALING

As Extracellular Glutamine Levels Decline, Asparagine Becomes an Essential Amino Acid

(2018) Natalya N. Pavlova et al.   Cell Metabolism

Spatiotemporal compartmentalization of hepatic NADH and NADPH metabolism

(2018) Russell P. Goodman et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Role of eIF2α Kinases in Translational Control and Adaptation to Cellular Stress

(2018) Ronald C. Wek   Cold Spring Harbor Perspectives in Biology

Inhibition of GCN2 sensitizes ASNS-low cancer cells to asparaginase by disrupting the amino acid response

(2018) Akito Nakamura et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Neuronal Mitochondrial Dysfunction Activates the Integrated Stress Response to Induce Fibroblast Growth Factor 21

(2018) Lisa Michelle Restelli et al.   Cell Reports

Heavy-tailed prior distributions for sequence count data: removing the noise and preserving large differences

(2018) Anqi Zhu et al.   BIOINFORMATICS

Mitochondrial energy generation disorders: genes, mechanisms and clues to pathology

(2017) Ann E. Frazier et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

Mitochondrial diseases: the contribution of organelle stress responses to pathology

(2017) Anu Suomalainen et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Mitochondrial respiratory gene expression is suppressed in many cancers

(2017) Ed Reznik et al.   eLife

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

(2017) Inge Kühl et al.   eLife

Coenzyme Q biosynthesis and its role in the respiratory chain structure

(2016) María Alcázar-Fabra et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Absolute Quantification of Matrix Metabolites Reveals the Dynamics of Mitochondrial Metabolism

(2016) Walter W. Chen et al.   CELL

Mitochondrial DNA Replication Defects Disturb Cellular dNTP Pools and Remodel One-Carbon Metabolism

(2016) Joni Nikkanen et al.   Cell Metabolism

The integrated stress response

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

Skeletal muscle–specific eukaryotic translation initiation factor 2α phosphorylation controls amino acid metabolism and fibroblast growth factor 21–mediated non–cell-autonomous energy metabolism

(2016) Masato Miyake et al.   FASEB JOURNAL

Mitochondrial generation of superoxide and hydrogen peroxide as the source of mitochondrial redox signaling

(2016) Martin D. Brand   FREE RADICAL BIOLOGY AND MEDICINE

Growth differentiation factor 15 is a myomitokine governing systemic energy homeostasis

(2016) Hyo Kyun Chung et al.   JOURNAL OF CELL BIOLOGY

Embryonic cardiomyocytes can orchestrate various cell protective mechanisms to survive mitochondrial stress

(2016) Manuela Magarin et al.   JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY

The Mitochondrial Basis of Aging

(2016) Nuo Sun et al.   MOLECULAR CELL

Near-optimal probabilistic RNA-seq quantification

(2016) Nicolas L Bray et al.   NATURE BIOTECHNOLOGY

Metabolite concentrations, fluxes and free energies imply efficient enzyme usage

(2016) Junyoung O Park et al.   Nature Chemical Biology

FGF21 is a biomarker for mitochondrial translation and mtDNA maintenance disorders

(2016) Jenni M. Lehtonen et al.   NEUROLOGY

Complementation of mitochondrial electron transport chain by manipulation of the NAD+/NADH ratio

(2016) D. V. Titov et al.   SCIENCE

Mitochondrial dysfunction remodels one-carbon metabolism in human cells

(2016) Xiaoyan Robert Bao et al.   eLife

Mitochondrial diseases

(2016) Gráinne S. Gorman et al.   Nature Reviews Disease Primers

Mitochondrial dysfunction in Parkinson’s disease

(2016) Qingsong Hu et al.   Translational Neurodegeneration

Natural Product Screening Reveals Naphthoquinone Complex I Bypass Factors

(2016) Scott B. Vafai et al.   PLoS One

Integrated stress response of vertebrates is regulated by four eIF2α kinases

(2016) Shusuke Taniuchi et al.   Scientific Reports

Prevalence of nuclear and mitochondrial DNA mutations related to adult mitochondrial disease

(2015) Gráinne S. Gorman et al.   ANNALS OF NEUROLOGY

Growth differentiation factor 15 as a useful biomarker for mitochondrial disorders

(2015) Shuichi Yatsuga et al.   ANNALS OF NEUROLOGY

Mitophagy is required for mitochondrial biogenesis and myogenic differentiation of C2C12 myoblasts

(2015) Jon Sin et al.   Autophagy

An Essential Role of the Mitochondrial Electron Transport Chain in Cell Proliferation Is to Enable Aspartate Synthesis

(2015) Kıvanç Birsoy et al.   CELL

Supporting Aspartate Biosynthesis Is an Essential Function of Respiration in Proliferating Cells

(2015) Lucas B. Sullivan et al.   CELL

The unpredictability of prolonged activation of stress response pathways

(2015) Lilian T. Lamech et al.   JOURNAL OF CELL BIOLOGY

GDF15 is a novel biomarker to evaluate efficacy of pyruvate therapy for mitochondrial diseases

(2015) Yasunori Fujita et al.   MITOCHONDRION

Keeping the eIF2 alpha kinase Gcn2 in check

(2014) Beatriz A. Castilho et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Statistical significance of variables driving systematic variation in high-dimensional data

(2014) Neo Christopher Chung et al.   BIOINFORMATICS

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

(2014) Sukru Anil Dogan et al.   Cell Metabolism

Stress-induced OMA1 activation and autocatalytic turnover regulate OPA1-dependent mitochondrial dynamics

(2014) M. J. Baker et al.   EMBO JOURNAL

Membrane depolarization activates the mitochondrial protease OMA1 by stimulating self-cleavage

(2014) K. Zhang et al.   EMBO REPORTS

Full-length RNA-seq from single cells using Smart-seq2

(2014) Simone Picelli et al.   Nature Protocols

A sustained deficiency of mitochondrial respiratory complex III induces an apoptotic cell death through the p53-mediated inhibition of pro-survival activities of the activating transcription factor 4

(2014) A G Evstafieva et al.   Cell Death & Disease

iRegulon: From a Gene List to a Gene Regulatory Network Using Large Motif and Track Collections

(2014) Rekin's Janky et al.   PLoS Computational Biology

Quantitative determinants of aerobic glycolysis identify flux through the enzyme GAPDH as a limiting step

(2014) Alexander A Shestov et al.   eLife

A note on the kinetics of enzyme action: A decomposition that highlights thermodynamic effects

(2013) Elad Noor et al.   FEBS LETTERS

Synthesis of Mitochondrial DNA Precursors during Myogenesis, an Analysis in Purified C2C12 Myotubes

(2013) Miriam Frangini et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mitochondrial complex I activity and NAD+/NADH balance regulate breast cancer progression

(2013) Antonio F. Santidrian et al.   JOURNAL OF CLINICAL INVESTIGATION

The role of mitochondria in aging

(2013) Ana Bratic et al.   JOURNAL OF CLINICAL INVESTIGATION

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

(2013) Jaeseok Han et al.   NATURE CELL BIOLOGY

The Reactome pathway knowledgebase

(2013) David Croft et al.   NUCLEIC ACIDS RESEARCH

Discovery of GSK2656157: An Optimized PERK Inhibitor Selected for Preclinical Development

(2013) Jeffrey M. Axten et al.   ACS Medicinal Chemistry Letters

Identification of a novel mitochondrial uncoupler that does not depolarize the plasma membrane

(2013) Brandon M. Kenwood et al.   Molecular Metabolism

The function and the role of the mitochondrial glycerol-3-phosphate dehydrogenase in mammalian tissues

(2012) Tomáš Mráček et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Mitochondrial diseases

(2012) Anthony HV Schapira   LANCET

Mitochondrial disorders as windows into an ancient organelle

(2012) Scott B. Vafai et al.   NATURE

Assessing mitochondrial dysfunction in cells

(2011) Martin D. Brand et al.   BIOCHEMICAL JOURNAL

Chemiosmotic coupling in oxidative and photosynthetic phosphorylation

(2011) Peter Mitchell   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Proteomics-based investigation in C2C12 myoblast differentiation

(2011) L. Casadei et al.   EUROPEAN JOURNAL OF HISTOCHEMISTRY

Phosphorylation of eIF2 Facilitates Ribosomal Bypass of an Inhibitory Upstream ORF to EnhanceCHOPTranslation

(2011) Lakshmi Reddy Palam et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

The Regulation and Physiology of Mitochondrial Proton Leak

(2011) Ajit S. Divakaruni et al.   PHYSIOLOGY

Hypoxia. 2. Hypoxia regulates cellular metabolism

(2010) William W. Wheaton et al.   AMERICAN JOURNAL OF PHYSIOLOGY-CELL PHYSIOLOGY

Hypoxia-inducible factor 1: Regulator of mitochondrial metabolism and mediator of ischemic preconditioning

(2010) Gregg L. Semenza   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Mitochondrial proton and electron leaks

(2010) Martin Jastroch et al.   Essays in Biochemistry

Mitochondrial myopathy induces a starvation-like response

(2010) Henna Tyynismaa et al.   HUMAN MOLECULAR GENETICS

Pyrimidine biosynthesis links mitochondrial respiration to the p53 pathway

(2010) A. A. Khutornenko et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Quiescent Fibroblasts Exhibit High Metabolic Activity

(2010) Johanna M. S. Lemons et al.   PLOS BIOLOGY

Deficiency of electron transport chain in human skeletal muscle mitochondria in type 2 diabetes mellitus and obesity

(2009) Vladimir B. Ritov et al.   AMERICAN JOURNAL OF PHYSIOLOGY-ENDOCRINOLOGY AND METABOLISM

Peculiar inhibition of human mitochondrial aspartyl-tRNA synthetaseby adenylate analogs

(2009) Marie Messmer et al.   BIOCHIMIE

Plasmodial Aspartyl-tRNA Synthetases and Peculiarities inPlasmodium falciparum

(2009) Tania Bour 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

Regulation of mitochondrial biogenesis during myogenesis

(2009) A.H.V. Remels et al.   MOLECULAR AND CELLULAR ENDOCRINOLOGY

Inhibition of mitochondrial function induces an integrated stress response in oligodendroglia

(2009) Jillian M. Silva et al.   NEUROBIOLOGY OF DISEASE

PGC-1  is coupled to HIF-1 -dependent gene expression by increasing mitochondrial oxygen consumption in skeletal muscle cells

(2009) K. A. O'Hagan et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Understanding the Warburg Effect: The Metabolic Requirements of Cell Proliferation

(2009) M. G. Vander Heiden et al.   SCIENCE

Amino-acid limitation induces the GCN2 signaling pathway in myoblasts but not in myotubes

(2008) Christiane Deval et al.   BIOCHIMIE

Mitochondria in the aetiology and pathogenesis of Parkinson's disease

(2008) Anthony HV Schapira   LANCET NEUROLOGY

NAD+/NADH and NADP+/NADPH in Cellular Functions and Cell Death: Regulation and Biological Consequences

(2007) Weihai Ying   ANTIOXIDANTS & REDOX SIGNALING