CO2-sensitive tRNA modification associated with human mitochondrial disease

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

(2018) Kana Asano et al.   NUCLEIC ACIDS RESEARCH

Defective Mitochondrial tRNA Taurine Modification Activates Global Proteostress and Leads to Mitochondrial Disease

(2018) Md Fakruddin et al.   Cell Reports

tRNA N6-adenosine threonylcarbamoyltransferase defect due to KAE1/TCS3 (OSGEP) mutation manifest by neurodegeneration and renal tubulopathy

(2017) Simon Edvardson et al.   EUROPEAN JOURNAL OF HUMAN GENETICS

Mutations in KEOPS-complex genes cause nephrotic syndrome with primary microcephaly

(2017) Daniela A Braun et al.   NATURE GENETICS

NSUN3 methylase initiates 5-formylcytidine biogenesis in human mitochondrial tRNAMet

(2016) Saori Nakano et al.   Nature Chemical Biology

RNA modifications: what have we learned and where are we headed?

(2016) Michaela Frye et al.   NATURE REVIEWS GENETICS

Identification of 2-methylthio cyclic N6-threonylcarbamoyladenosine (ms2ct6A) as a novel RNA modification at position 37 of tRNAs

(2016) Byeong-il Kang et al.   NUCLEIC ACIDS RESEARCH

A hydantoin isoform of cyclic N6-threonylcarbamoyladenosine (ct6A) is present in tRNAs

(2016) Michal Matuszewski et al.   NUCLEIC ACIDS RESEARCH

Optimization of Codon Translation Rates via tRNA Modifications Maintains Proteome Integrity

(2015) Danny D. Nedialkova et al.   CELL

The ATP-mediated formation of the YgjD–YeaZ–YjeE complex is required for the biosynthesis of tRNA t6A in Escherichia coli

(2015) Wenhua Zhang et al.   NUCLEIC ACIDS RESEARCH

Single methylation of 23S rRNA triggers late steps of 50S ribosomal subunit assembly

(2015) Taiga Arai et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

Cross Kingdom Functional Conservation of the Core Universally Conserved Threonylcarbamoyladenosine tRNA Synthesis Enzymes

(2014) Patrick C. Thiaville et al.   EUKARYOTIC CELL

Discovery of the β-barrel–type RNA methyltransferase responsible forN6-methylation ofN6-threonylcarbamoyladenosine in tRNAs

(2014) Satoshi Kimura et al.   NUCLEIC ACIDS RESEARCH

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

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

Comprehensive next-generation sequence analyses of the entire mitochondrial genome reveal new insights into the molecular diagnosis of mitochondrial DNA disorders

(2013) Hong Cui et al.   GENETICS IN MEDICINE

Juvenile parkinsonism, hypogonadism and Leigh-like MRI changes in a patient with m.4296G>A mutation in mitochondrial DNA

(2013) Mika H. Martikainen et al.   MITOCHONDRION

Functional assignment of KEOPS/EKC complex subunits in the biosynthesis of the universal t 6 A tRNA modification

(2013) Ludovic Perrochia et al.   NUCLEIC ACIDS RESEARCH

Reconstitution and characterization of eukaryotic N6-threonylcarbamoylation of tRNA using a minimal enzyme system

(2013) Leo C. K. Wan et al.   NUCLEIC ACIDS RESEARCH

Multiplex Genome Engineering Using CRISPR/Cas Systems

(2013) L. Cong et al.   SCIENCE

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

Mechanism of N6-Threonylcarbamoyladenonsine (t6A) Biosynthesis: Isolation and Characterization of the Intermediate Threonylcarbamoyl-AMP

(2012) Charles T. Lauhon   BIOCHEMISTRY

Measuring Equilibrium Bicarbonate Concentrations Directly in Cellular Mitochondria and in Human Serum Using Europium/Terbium Emission Intensity Ratios

(2012) David G Smith et al.   CHEMISTRY-A EUROPEAN JOURNAL

Biosynthesis of Threonylcarbamoyl Adenosine (t6A), a Universal tRNA Nucleoside

(2012) Christopher Deutsch et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A cyclic form of N6-threonylcarbamoyladenosine as a widely distributed tRNA hypermodification

(2012) Kenjyo Miyauchi et al.   Nature Chemical Biology

MODOMICS: a database of RNA modification pathways—2013 update

(2012) Magdalena A. Machnicka et al.   NUCLEIC ACIDS RESEARCH

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

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

A role for the universal Kae1/Qri7/YgjD (COG0533) family in tRNA modification

(2011) Basma El Yacoubi et al.   EMBO JOURNAL

Kinetic investigation on carbamate formation from the reaction of carbon dioxide with amino acids in homogeneous aqueous solution

(2011) Yuki Yamamoto et al.   JOURNAL OF PHYSICAL ORGANIC CHEMISTRY

Leigh syndrome caused by a novel m.4296G>A mutation in mitochondrial tRNA isoleucine

(2011) Rachel Cox et al.   MITOCHONDRION

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

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

The highly conserved KEOPS/EKC complex is essential for a universal tRNA modification, t6A

(2010) Madhusudhan Srinivasan et al.   EMBO JOURNAL

Identification of Eukaryotic and Prokaryotic Methylthiotransferase for Biosynthesis of 2-Methylthio-N6-threonylcarbamoyladenosine in tRNA

(2010) Simon Arragain et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Acute Infantile Liver Failure Due to Mutations in the TRMU Gene

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

Eukaryotic GCP1 is a conserved mitochondrial protein required for progression of embryo development beyond the globular stage inArabidopsis thaliana

(2009) Kirsten Haussuehl et al.   BIOCHEMICAL JOURNAL

The Sua5 Protein Is Essential for Normal Translational Regulation in Yeast

(2009) C. A. Lin et al.   MOLECULAR AND CELLULAR BIOLOGY

Qri7/OSGEPL, the mitochondrial version of the universal Kae1/YgjD protein, is essential for mitochondrial genome maintenance

(2009) Jacques Oberto et al.   NUCLEIC ACIDS RESEARCH

Understanding the Warburg Effect: The Metabolic Requirements of Cell Proliferation

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

tRNAdb 2009: compilation of tRNA sequences and tRNA genes

(2008) F. Juhling et al.   NUCLEIC ACIDS RESEARCH