Defects in the mitochondrial-tRNA modification enzymes MTO1 and GTPBP3 promote different metabolic reprogramming through a HIF-PPARγ-UCP2-AMPK axis

A compendium of proteins that interact with HIF-1α

(2017) Gregg L. Semenza   EXPERIMENTAL CELL RESEARCH

Mutations in the Caenorhabditis elegans orthologs of human genes required for mitochondrial tRNA modification cause similar electron transport chain defects but different nuclear responses

(2017) Carmen Navarro-González et al.   PLoS Genetics

Measurement of β-oxidation capacity of biological samples by respirometry: a review of principles and substrates

(2016) Edward Ojuka et al.   AMERICAN JOURNAL OF PHYSIOLOGY-ENDOCRINOLOGY AND METABOLISM

Lipid metabolism and signaling in cardiac lipotoxicity

(2016) Kenneth D'Souza et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS

Peroxisome proliferator-activated receptors in cardiac energy metabolism and cardiovascular disease

(2016) Thekkuttuparambil Ananthanarayanan Ajith et al.   CLINICAL AND EXPERIMENTAL PHARMACOLOGY AND PHYSIOLOGY

The homozygous R504C mutation inMTO1gene is responsible for ONCE syndrome

(2016) M.Á. Martín et al.   CLINICAL GENETICS

CLPP coordinates mitoribosomal assembly through the regulation of ERAL1 levels

(2016) Karolina Szczepanowska et al.   EMBO JOURNAL

Loss of CLPP alleviates mitochondrial cardiomyopathy without affecting the mammalian UPRmt

(2016) Dominic Seiferling et al.   EMBO REPORTS

Uncoupling protein 2 downregulation by hypoxia through repression of peroxisome proliferator-activated receptor γ promotes chemoresistance of non-small cell lung cancer

(2016) Mingxing Wang et al.   Oncotarget

Mtu1-Mediated Thiouridine Formation of Mitochondrial tRNAs Is Required for Mitochondrial Translation and Is Involved in Reversible Infantile Liver Injury

(2016) Yong Wu et al.   PLoS Genetics

Optic neuropathy, cardiomyopathy, cognitive disability in patients with a homozygous mutation in the nuclearMTO1and a mitochondrialMT-TFvariant

(2015) Majida Charif et al.   AMERICAN JOURNAL OF MEDICAL GENETICS PART A

Mitochondrial Protein Quality Control: The Mechanisms Guarding Mitochondrial Health

(2015) Iryna Bohovych et al.   ANTIOXIDANTS & REDOX SIGNALING

DEC1 negatively regulates AMPK activity via LKB1

(2015) Fuyuki Sato et al.   BIOCHEMICAL AND BIOPHYSICAL RESEARCH COMMUNICATIONS

Quality control of mitochondrial protein synthesis is required for membrane integrity and cell fitness

(2015) Uwe Richter et al.   JOURNAL OF CELL BIOLOGY

Mitochondrial transcript maturation and its disorders

(2015) Lindsey Van Haute et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Cancer metabolism and the Warburg effect: the role of HIF-1 and PI3K

(2015) Rupert Courtnay et al.   MOLECULAR BIOLOGY REPORTS

New roles for mitochondrial proteases in health, ageing and disease

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

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

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

(2015) Ana Martínez-Zamora et al.   PLoS One

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

Mitochondrial Retrograde Signaling Mediated by UCP2 Inhibits Cancer Cell Proliferation and Tumorigenesis

(2014) P. Esteves et al.   CANCER RESEARCH

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

A deafness-associated tRNAHismutation alters the mitochondrial function, ROS production and membrane potential

(2014) Shasha Gong et al.   NUCLEIC ACIDS RESEARCH

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

Mitochondrial respiratory chain complex I is inactivated by NADPH oxidase Nox4

(2013) Rafał Kozieł et al.   BIOCHEMICAL JOURNAL

UCP2, a mitochondrial protein regulated at multiple levels

(2013) Massimo Donadelli et al.   CELLULAR AND MOLECULAR LIFE SCIENCES

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

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

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

Targeting mitochondrial oxidative metabolism as an approach to treat heart failure

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

Lipid Metabolism and Toxicity in the Heart

(2012) Ira J. Goldberg et al.   Cell Metabolism

AMPK Is a Negative Regulator of the Warburg Effect and Suppresses Tumor Growth In Vivo

(2012) Brandon Faubert et al.   Cell Metabolism

The updated biology of hypoxia-inducible factor

(2012) Samantha N Greer et al.   EMBO JOURNAL

AFG3L2 supports mitochondrial protein synthesis and Purkinje cell survival

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

UCP2 regulates energy metabolism and differentiation potential of human pluripotent stem cells

(2011) Jin Zhang et al.   EMBO JOURNAL

The nuclear receptor PPAR /  programs muscle glucose metabolism in cooperation with AMPK and MEF2

(2011) Z. Gan et al.   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

Functional regulation of HIF-1α under normoxia-is there more than post-translational regulation?

(2011) A. Kuschel et al.   JOURNAL OF CELLULAR PHYSIOLOGY

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

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

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

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

Acute Infantile Liver Failure Due to Mutations in the TRMU Gene

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

HIF-1: upstream and downstream of cancer metabolism

(2009) Gregg L Semenza   CURRENT OPINION IN GENETICS & DEVELOPMENT

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

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

Combination of the Loss of cmnm5U34 with the Lack of s2U34 Modifications of tRNALys, tRNAGlu, and tRNAGln Altered Mitochondrial Biogenesis and Respiration

(2009) Xinjian Wang et al.   JOURNAL OF MOLECULAR 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

Conserved Cysteine Residues of GidA Are Essential for Biogenesis of 5-Carboxymethylaminomethyluridine at tRNA Anticodon

(2009) Takuo Osawa et al.   STRUCTURE

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

Prostaglandin F2α induces the normoxic activation of the hypoxia-inducible factor-1 transcription factor in differentiating 3T3-L1 preadipocytes: Potential role in the regulation of adipogenesis

(2008) Li Liu et al.   JOURNAL OF CELLULAR BIOCHEMISTRY

Stra13/DEC1 and DEC2 inhibit sterol regulatory element binding protein-1c in a hypoxia-inducible factor-dependent mechanism

(2008) Su Mi Choi et al.   NUCLEIC ACIDS RESEARCH

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

(2007) Claire Pecqueur et al.   FASEB JOURNAL