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

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

Integrated transcriptome analysis across mitochondrial disease etiologies and tissues improves understanding of common cellular adaptations to respiratory chain dysfunction

(2014) Zhe Zhang et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Mitochondria: Impaired mitochondrial translation in human disease

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

Retracted: Mitochondrial protein acetylation mediates nutrient sensing of mitochondrial protein synthesis and mitonuclear protein balance

(2014) Antonella Di Domenico et al.   IUBMB LIFE

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

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

Amino Acid Starvation Has Opposite Effects on Mitochondrial and Cytosolic Protein Synthesis

(2014) Mark A. Johnson et al.   PLoS One

MTO1-Deficient Mouse Model Mirrors the Human Phenotype Showing Complex I Defect and Cardiomyopathy

(2014) Lore Becker et al.   PLoS One

Unsolved issues related to human mitochondrial diseases

(2013) Anne Lombès et al.   BIOCHIMIE

Defining the action spectrum of potential PGC-1α activators on a mitochondrial and cellular level in vivo

(2013) Annette Hofer et al.   HUMAN MOLECULAR GENETICS

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

Mitochondrial aminoacyl-tRNA synthetases in human disease

(2013) Svetlana Konovalova et al.   MOLECULAR GENETICS AND METABOLISM

Mitonuclear protein imbalance as a conserved longevity mechanism

(2013) Riekelt H. Houtkooper et al.   NATURE

tRNA tKUUU, tQUUG, and tEUUC wobble position modifications fine-tune protein translation by promoting ribosome A-site binding

(2013) V. A. N. Rezgui et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

mTOR Inhibition Alleviates Mitochondrial Disease in a Mouse Model of Leigh Syndrome

(2013) S. C. Johnson et al.   SCIENCE

Nutrient Signaling in Protein Homeostasis: An Increase in Quantity at the Expense of Quality

(2013) C. S. Conn et al.   Science Signaling

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

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

A Role for tRNA Modifications in Genome Structure and Codon Usage

(2012) Eva Maria Novoa et al.   CELL

CRIF1 Is Essential for the Synthesis and Insertion of Oxidative Phosphorylation Polypeptides in the Mammalian Mitochondrial Membrane

(2012) Soung Jung Kim et al.   Cell Metabolism

Nonsense mutations in the COX1 subunit impair the stability of respiratory chain complexes rather than their assembly

(2012) Hue-Tran Hornig-Do et al.   EMBO JOURNAL

RETRACTED: Bezafibrate improves mitochondrial function in the CNS of a mouse model of mitochondrial encephalopathy

(2012) Natalie Noe et al.   MITOCHONDRION

NIH Image to ImageJ: 25 years of image analysis

(2012) Caroline A Schneider et al.   NATURE METHODS

Amino acid sensing and regulation of mTORC1

(2012) Lijun Yan et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

Differing effects of rapamycin and mTOR kinase inhibitors on protein synthesis

(2011) Yilin Huo et al.   BIOCHEMICAL SOCIETY TRANSACTIONS

Human diseases with impaired mitochondrial protein synthesis

(2011) Agnès Rötig   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Multitasking in the mitochondrion by the ATP-dependent Lon protease

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

The ketogenic diet inhibits the mammalian target of rapamycin (mTOR) pathway

(2011) Sharon S. McDaniel et al.   EPILEPSIA

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

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

Determinants of translation efficiency and accuracy

(2011) H. Gingold et al.   Molecular Systems Biology

A functional peptidyl-tRNA hydrolase, ICT1, has been recruited into the human mitochondrial ribosome

(2010) Ricarda Richter et al.   EMBO JOURNAL

Ketogenic diet slows down mitochondrial myopathy progression in mice

(2010) Sofia Ahola-Erkkilä et al.   HUMAN MOLECULAR GENETICS

A metabolic shift induced by a PPAR panagonist markedly reduces the effects of pathogenic mitochondrial tRNA mutations

(2010) Tina Wenz et al.   JOURNAL OF CELLULAR AND MOLECULAR MEDICINE

RETRACTED: mTERF2 Regulates Oxidative Phosphorylation by Modulating mtDNA Transcription

(2009) Tina Wenz et al.   Cell Metabolism

Structure-Function Analysis of Escherichia coli MnmG (GidA), a Highly Conserved tRNA-Modifying Enzyme

(2009) R. Shi et al.   JOURNAL OF BACTERIOLOGY

G-Domain Dimerization Orchestrates the tRNA Wobble Modification Reaction in the MnmE/GidA Complex

(2009) Simon Meyer et al.   JOURNAL OF MOLECULAR BIOLOGY

Mutation in MTO1 involved in tRNA modification impairs mitochondrial RNA metabolism in the yeast Saccharomyces cerevisiae

(2009) Xinjian Wang et al.   MITOCHONDRION

Mitochondrial Disorders in the Nervous System

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

The A3243G tRNALeu(UUR) MELAS mutation causes amino acid misincorporation and a combined respiratory chain assembly defect partially suppressed by overexpression of EFTu and EFG2

(2008) F. Sasarman et al.   HUMAN MOLECULAR GENETICS