Deletion of Gtpbp3 in zebrafish revealed the hypertrophic cardiomyopathy manifested by aberrant mitochondrial tRNA metabolism

Leber’s hereditary optic neuropathy (LHON)-associated ND5 12338T > C mutation altered the assembly and function of complex I, apoptosis and mitophagy

(2018) Juanjuan Zhang et al.   HUMAN MOLECULAR GENETICS

Metabolic and chemical regulation of tRNA modification associated with taurine deficiency and human disease

(2018) Kana Asano et al.   NUCLEIC ACIDS RESEARCH

Phenotyping cardiomyopathy in adult zebrafish

(2018) Alexey V. Dvornikov et al.   PROGRESS IN BIOPHYSICS & MOLECULAR BIOLOGY

A coronary artery disease-associated tRNAThr mutation altered mitochondrial function, apoptosis and angiogenesis

(2018) Zidong Jia et al.   NUCLEIC ACIDS RESEARCH

A hypertension-associated mitochondrial DNA mutation introduces an m1G37 modification into tRNAMet, altering its structure and function

(2017) Mi Zhou et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Biochemical Evidence for a Nuclear Modifier Allele (A10S) in TRMU (Methylaminomethyl-2-thiouridylate-methyltransferase) Related to Mitochondrial tRNA Modification in the Phenotypic Manifestation of Deafness-associated 12S rRNA Mutation

(2017) Feilong Meng et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

MODOMICS: a database of RNA modification pathways. 2017 update

(2017) Pietro Boccaletto et al.   NUCLEIC ACIDS RESEARCH

The RNA modification landscape in human disease

(2017) Nicky Jonkhout et al.   RNA

CLPP coordinates mitoribosomal assembly through the regulation of ERAL1 levels

(2016) Karolina Szczepanowska et al.   EMBO JOURNAL

Development of the cardiac conduction system in zebrafish

(2016) Kar-Lai Poon et al.   GENE EXPRESSION PATTERNS

The defective expression of gtpbp3 related to tRNA modification alters the mitochondrial function and development of zebrafish

(2016) Danni Chen et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

A Deafness- and Diabetes-associated tRNA Mutation Causes Deficient Pseudouridinylation at Position 55 in tRNAGluand Mitochondrial Dysfunction

(2016) Meng Wang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Differential Lectin Binding Patterns Identify Distinct Heart Regions in Giant Danio (Devario aequipinnatus) and Zebrafish (Danio rerio) Hearts

(2016) Trina Manalo et al.   JOURNAL OF HISTOCHEMISTRY & CYTOCHEMISTRY

A Hypertension-Associated tRNAAlaMutation Alters tRNA Metabolism and Mitochondrial Function

(2016) Pingping Jiang et al.   MOLECULAR AND CELLULAR BIOLOGY

NSUN3 methylase initiates 5-formylcytidine biogenesis in human mitochondrial tRNAMet

(2016) Saori Nakano et al.   Nature Chemical Biology

A deafness-associated tRNAAspmutation alters the m1G37 modification, aminoacylation and stability of tRNAAspand mitochondrial function

(2016) Meng Wang et al.   NUCLEIC ACIDS RESEARCH

Translation quality control is critical for bacterial responses to amino acid stress

(2016) Tammy J. Bullwinkle et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Small RNA Modifications: Integral to Function and Disease

(2016) Xudong Zhang et al.   TRENDS IN MOLECULAR MEDICINE

Deficient methylation and formylation of mt-tRNAMet wobble cytosine in a patient carrying mutations in NSUN3

(2016) Lindsey Van Haute et al.   Nature Communications

A Comprehensive Genomic Analysis Reveals the Genetic Landscape of Mitochondrial Respiratory Chain Complex Deficiencies

(2016) Masakazu Kohda et al.   PLoS Genetics

Downregulation of GSTK1 Is a Common Mechanism Underlying Hypertrophic Cardiomyopathy

(2016) Shota Sasagawa et al.   Frontiers in Pharmacology

Optimization of Codon Translation Rates via tRNA Modifications Maintains Proteome Integrity

(2015) Danny D. Nedialkova et al.   CELL

The role of wobble uridine modifications in +1 translational frameshifting in eukaryotes

(2015) Hasan Tükenmez et al.   NUCLEIC ACIDS RESEARCH

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

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

(2014) Sukru Anil Dogan et al.   Cell Metabolism

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

Leber's hereditary optic neuropathy caused by the homoplasmic ND1 m.3635G>A mutation in nine Han Chinese families

(2014) Juanjuan Zhang et al.   MITOCHONDRION

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

TRNA mutations that affect decoding fidelity deregulate development and the proteostasis network in zebrafish

(2014) Marisa Reverendo et al.   RNA Biology

Multilevel functional and structural defects induced by two pathogenic mitochondrial tRNA mutations

(2013) Meng Wang et al.   BIOCHEMICAL JOURNAL

Differential activation of natriuretic peptide receptors modulates cardiomyocyte proliferation during development

(2013) J. R. Becker et al.   DEVELOPMENT

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

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

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

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

Biosynthesis and Function of Posttranscriptional Modifications of Transfer RNAs

(2012) Basma El Yacoubi et al.   Annual Review of Genetics

Enzymology of tRNA modification in the bacterial MnmEG pathway

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

Hypertrophic cardiomyopathy

(2012) Barry J Maron et al.   LANCET

NIH Image to ImageJ: 25 years of image analysis

(2012) Caroline A Schneider et al.   NATURE METHODS

Human mitochondrial tRNA quality control in health and disease

(2012) Ruth Belostotsky et al.   RNA Biology

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

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

Analysis of the Mitochondrial Complex I-V Enzyme Activities of Peripheral Leukocytes in Oxidative Phosphorylation Disorders

(2011) Yan-Yan Ma et al.   JOURNAL OF CHILD NEUROLOGY

Determinants of translation efficiency and accuracy

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

tRNA biology charges to the front

(2010) E. M. Phizicky et al.   GENES & DEVELOPMENT

The RNA modification database, RNAMDB: 2011 update

(2010) W. A. Cantara et al.   NUCLEIC ACIDS RESEARCH

Acute Infantile Liver Failure Due to Mutations in the TRMU Gene

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

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

Tertiary structure checkpoint at anticodon loop modification in tRNA functional maturation

(2009) Sakurako Goto-Ito et al.   NATURE STRUCTURAL & 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

Modified Uridines with C5-methylene Substituents at the First Position of the tRNA Anticodon Stabilize U·G Wobble Pairing during Decoding

(2008) Shinya Kurata et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

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

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