Defective Expression of the Mitochondrial-tRNA Modifying Enzyme GTPBP3 Triggers AMPK-Mediated Adaptive Responses Involving Complex I Assembly Factors, Uncoupling Protein 2, and the Mitochondrial Pyruvate Carrier

MTO1 mediates tissue specificity of OXPHOS defects via tRNA modification and translation optimization, which can be bypassed by dietary intervention

(2015) Christin Tischner et al.   HUMAN MOLECULAR GENETICS

Modification of the wobble uridine in bacterial and mitochondrial tRNAs reading NNA/NNG triplets of 2-codon boxes

(2015) M Eugenia Armengod et al.   RNA Biology

Mutations in GTPBP3 Cause a Mitochondrial Translation Defect Associated with Hypertrophic Cardiomyopathy, Lactic Acidosis, and Encephalopathy

(2014) Robert Kopajtich et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Aberrant methylation of tRNAs links cellular stress to neuro-developmental disorders

(2014) S. Blanco et al.   EMBO JOURNAL

The ROS-sensitive microRNA-9/9* controls the expression of mitochondrial tRNA-modifying enzymes and is involved in the molecular mechanism of MELAS syndrome

(2014) Salvador Meseguer et al.   HUMAN MOLECULAR GENETICS

Mitochondria: Impaired mitochondrial translation in human disease

(2014) Veronika Boczonadi et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Use of Whole-Exome Sequencing to Determine the Genetic Basis of Multiple Mitochondrial Respiratory Chain Complex Deficiencies

(2014) Robert W. Taylor et al.   JAMA-JOURNAL OF THE AMERICAN MEDICAL ASSOCIATION

Role of Metabolic H2O2Generation

(2014) Helmut Sies   JOURNAL OF BIOLOGICAL CHEMISTRY

Glutamine Oxidation Maintains the TCA Cycle and Cell Survival during Impaired Mitochondrial Pyruvate Transport

(2014) Chendong Yang et al.   MOLECULAR CELL

Regulation of Substrate Utilization by the Mitochondrial Pyruvate Carrier

(2014) Nathaniel M. Vacanti et al.   MOLECULAR CELL

A complete landscape of post-transcriptional modifications in mammalian mitochondrial tRNAs

(2014) T. Suzuki et al.   NUCLEIC ACIDS RESEARCH

UCP2 transports C4 metabolites out of mitochondria, regulating glucose and glutamine oxidation

(2014) A. Vozza et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Loss of UCP2 Attenuates Mitochondrial Dysfunction without Altering ROS Production and Uncoupling Activity

(2014) Alexandra Kukat et al.   PLoS Genetics

The function of the respiratory supercomplexes: The plasticity model

(2013) Rebeca Acin-Perez et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Role of AMPK-mediated adaptive responses in human cells with mitochondrial dysfunction to oxidative stress

(2013) Shi-Bei Wu et al.   BIOCHIMICA ET BIOPHYSICA ACTA-GENERAL SUBJECTS

Regulation of pyruvate metabolism and human disease

(2013) Lawrence R. Gray et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

Cellular metabolic and autophagic pathways: Traffic control by redox signaling

(2013) Matthew Dodson et al.   FREE RADICAL BIOLOGY AND MEDICINE

Altered 2-thiouridylation impairs mitochondrial translation in reversible infantile respiratory chain deficiency

(2013) Veronika Boczonadi et al.   HUMAN MOLECULAR GENETICS

MTO1 Mutations are Associated with Hypertrophic Cardiomyopathy and Lactic Acidosis and Cause Respiratory Chain Deficiency in Humans and Yeast

(2013) Enrico Baruffini et al.   HUMAN MUTATION

AMPK-Dependent Degradation of TXNIP upon Energy Stress Leads to Enhanced Glucose Uptake via GLUT1

(2013) Ning Wu et al.   MOLECULAR CELL

The output of the tRNA modification pathways controlled by the Escherichia coli MnmEG and MnmC enzymes depends on the growth conditions and the tRNA species

(2013) Ismaïl Moukadiri et al.   NUCLEIC ACIDS RESEARCH

The Phenotype of Many Independently Isolated +1 Frameshift Suppressor Mutants Supports a Pivotal Role of the P-Site in Reading Frame Maintenance

(2013) Gunilla Jäger et al.   PLoS One

Tissue- and cell-type-specific manifestations of heteroplasmic mtDNA 3243A>G mutation in human induced pluripotent stem cell-derived disease model

(2013) R. H. Hamalainen et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

A Systems Approach for Decoding Mitochondrial Retrograde Signaling Pathways

(2013) S. Chae et al.   Science Signaling

Mitochondrial Biogenesis through Activation of Nuclear Signaling Proteins

(2013) J. E. Dominy et al.   Cold Spring Harbor Perspectives in Biology

Loss of a Conserved tRNA Anticodon Modification Perturbs Cellular Signaling

(2013) Boris Zinshteyn et al.   PLoS Genetics

Mutations of the Mitochondrial-tRNA Modifier MTO1 Cause Hypertrophic Cardiomyopathy and Lactic Acidosis

(2012) Daniele Ghezzi et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Mitochondrial (‘mild’) uncoupling and ROS production: physiologically relevant or not?

(2012) Irina G. Shabalina et al.   BIOCHEMICAL SOCIETY TRANSACTIONS

Targeting mitochondrial oxidative metabolism as an approach to treat heart failure

(2012) Natasha Fillmore et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Enzymology of tRNA modification in the bacterial MnmEG pathway

(2012) M.-Eugenia Armengod et al.   BIOCHIMIE

Mitochondria: In Sickness and in Health

(2012) Jodi Nunnari et al.   CELL

Early complex I assembly defects result in rapid turnover of the ND1 subunit

(2012) O. Zurita Rendon et al.   HUMAN MOLECULAR GENETICS

Mitochondria and cell signalling

(2012) Stephen W. G. Tait et al.   JOURNAL OF CELL SCIENCE

Transcriptional integration of mitochondrial biogenesis

(2012) Richard C. Scarpulla et al.   TRENDS IN ENDOCRINOLOGY AND METABOLISM

Human Mitochondrial tRNAs: Biogenesis, Function, Structural Aspects, and Diseases

(2011) Tsutomu Suzuki et al.   Annual Review of Genetics

Mitochondrial protection by the thioredoxin-2 and glutathione systems in anin vitroendothelial model of sepsis

(2011) Damon A. Lowes et al.   BIOCHEMICAL JOURNAL

Understanding mitochondrial complex I assembly in health and disease

(2011) Masakazu Mimaki et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Targeting fatty acid and carbohydrate oxidation — A novel therapeutic intervention in the ischemic and failing heart

(2011) Jagdip S. Jaswal et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Secondary coenzyme Q10 deficiency triggers mitochondria degradation by mitophagy in MELAS fibroblasts

(2011) David Cotán et al.   FASEB JOURNAL

AMP-activated protein kinase--an energy sensor that regulates all aspects of cell function

(2011) D. G. Hardie   GENES & DEVELOPMENT

The 2-thiouridylase function of the human MTU1 (TRMU) enzyme is dispensable for mitochondrial translation

(2011) F. Sasarman et al.   HUMAN MOLECULAR GENETICS

Emerging role of angiogenin in stress response and cell survival under adverse conditions

(2011) Shuping Li et al.   JOURNAL OF CELLULAR PHYSIOLOGY

Reversible infantile respiratory chain deficiency is a unique, genetically heterogenous mitochondrial disease

(2011) J. Uusimaa et al.   JOURNAL OF MEDICAL GENETICS

The AMPK signalling pathway coordinates cell growth, autophagy and metabolism

(2011) Maria M. Mihaylova et al.   NATURE CELL BIOLOGY

Deep-sequencing of human Argonaute-associated small RNAs provides insight into miRNA sorting and reveals Argonaute association with RNA fragments of diverse origin

(2011) Alexander Maxwell Burroughs et al.   RNA Biology

Evolution Meets Disease: Penetrance and Functional Epistasis of Mitochondrial tRNA Mutations

(2011) Raquel Moreno-Loshuertos et al.   PLoS Genetics

Human mitochondrial diseases caused by lack of taurine modification in mitochondrial tRNAs

(2011) Suzuki Tsutomu et al.   Wiley Interdisciplinary Reviews-RNA

The Interplay Between Mitochondrial Dynamics and Mitophagy

(2010) Gilad Twig et al.   ANTIOXIDANTS & REDOX SIGNALING

Mitochondrial fatty acid oxidation and oxidative stress: Lack of reverse electron transfer-associated production of reactive oxygen species

(2010) Peter Schönfeld et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Mitochondrial bioenergetics and dynamics interplay in complex I-deficient fibroblasts

(2010) M. Morán et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

Oxidative stress in skin fibroblasts cultures from patients with Parkinson's disease

(2010) Pilar del Hoyo et al.   BMC Neurology

Nuclear factors involved in mitochondrial translation cause a subgroup of combined respiratory chain deficiency

(2010) John P. Kemp et al.   BRAIN

AMP-activated protein kinase and its downstream transcriptional pathways

(2010) Carles Cantó et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

AMPK inhibition in health and disease

(2010) Benoit Viollet et al.   CRITICAL REVIEWS IN BIOCHEMISTRY AND MOLECULAR BIOLOGY

RNA methylation by Dnmt2 protects transfer RNAs against stress-induced cleavage

(2010) M. Schaefer et al.   GENES & DEVELOPMENT

Evidence for Physical Association of Mitochondrial Fatty Acid Oxidation and Oxidative Phosphorylation Complexes

(2010) Yudong Wang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mitochondrial Translation and Beyond: Processes Implicated in Combined Oxidative Phosphorylation Deficiencies

(2010) Paulien Smits et al.   JOURNAL OF BIOMEDICINE AND BIOTECHNOLOGY

Acute liver failure with subsequent cirrhosis as the primary manifestation of TRMU mutations

(2010) Ulrike Schara et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Hungry Codons Promote Frameshifting in Human Mitochondrial Ribosomes

(2010) R. Temperley et al.   SCIENCE

Acute Infantile Liver Failure Due to Mutations in the TRMU Gene

(2009) Avraham Zeharia et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Mutations in NDUFAF3 (C3ORF60), Encoding an NDUFAF4 (C6ORF66)-Interacting Complex I Assembly Protein, Cause Fatal Neonatal Mitochondrial Disease

(2009) Ann Saada et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Mitochondrial peroxiredoxin involvement in antioxidant defence and redox signalling

(2009) Andrew G. Cox et al.   BIOCHEMICAL JOURNAL

UCP2, not a physiologically relevant uncoupler but a glucose sparing switch impacting ROS production and glucose sensing

(2009) Frédéric Bouillaud   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Mitochondria as ATP consumers in cellular pathology

(2009) Christos Chinopoulos et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR BASIS OF DISEASE

EFNS guidelines on the molecular diagnosis of mitochondrial disorders

(2009) J. Finsterer et al.   EUROPEAN JOURNAL OF NEUROLOGY

UCP2, a metabolic sensor coupling glucose oxidation to mitochondrial metabolism?

(2009) Claire Pecqueur et al.   IUBMB LIFE

Angiogenin cleaves tRNA and promotes stress-induced translational repression

(2009) Satoshi Yamasaki et al.   JOURNAL OF CELL BIOLOGY

Evolutionarily conserved proteins MnmE and GidA catalyze the formation of two methyluridine derivatives at tRNA wobble positions

(2009) Ismaïl Moukadiri et al.   NUCLEIC ACIDS RESEARCH

Mitochondrial Disorders in the Nervous System

(2008) Salvatore DiMauro et al.   Annual Review of Neuroscience

Upregulation of Mitochondrial Uncoupling Protein-2 by the AMP-Activated Protein Kinase in Endothelial Cells Attenuates Oxidative Stress in Diabetes

(2008) Z. Xie et al.   DIABETES

Stress induces tRNA cleavage by angiogenin in mammalian cells

(2008) Hanjiang Fu et al.   FEBS LETTERS

Handling mammalian mitochondrial tRNAs and aminoacyl-tRNA synthetases for functional and structural characterization

(2008) Marie Sissler et al.   METHODS

Characterization of Human GTPBP3, a GTP-Binding Protein Involved in Mitochondrial tRNA Modification

(2008) M. Villarroya et al.   MOLECULAR AND CELLULAR BIOLOGY

Uncoupling protein-2 controls proliferation by promoting fatty acid oxidation and limiting glycolysis-derived pyruvate utilization

(2007) Claire Pecqueur et al.   FASEB JOURNAL