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

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 Hypertension-Associated tRNAAlaMutation Alters tRNA Metabolism and Mitochondrial Function

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

Cdk5rap1-Mediated 2-Methylthio Modification of Mitochondrial tRNAs Governs Protein Translation and Contributes to Myopathy in Mice and Humans

(2015) Fan-Yan Wei et al.   Cell Metabolism

Oxidative stress in inherited mitochondrial diseases

(2015) Genki Hayashi et al.   FREE RADICAL BIOLOGY AND MEDICINE

The exome sequencing identified the mutation in YARS2 encoding the mitochondrial tyrosyl-tRNA synthetase as a nuclear modifier for the phenotypic manifestation of Leber's hereditary optic neuropathy-associated mitochondrial DNA mutation

(2015) Pingping Jiang et al.   HUMAN MOLECULAR GENETICS

Mitochondrial tRNASer(UCN) variants in 2651 Han Chinese subjects with hearing loss

(2015) Xiaowen Tang et al.   MITOCHONDRION

Maternally inherited diabetes is associated with a homoplasmic T10003C mutation in the mitochondrial tRNAGly gene

(2015) Hao Liu et al.   MITOCHONDRION

Mutations of Human NARS2, Encoding the Mitochondrial Asparaginyl-tRNA Synthetase, Cause Nonsyndromic Deafness and Leigh Syndrome

(2015) Mariella Simon et al.   PLoS Genetics

A peep into mitochondrial disorder: multifaceted from mitochondrial DNA mutations to nuclear gene modulation

(2015) Chao Chen et al.   Protein & Cell

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

ROS Function in Redox Signaling and Oxidative Stress

(2014) Michael Schieber et al.   CURRENT BIOLOGY

Mitochondrial Genetics and Disease

(2014) Estela Area-Gomez et al.   JOURNAL OF CHILD NEUROLOGY

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

Mutations in LARS2, Encoding Mitochondrial Leucyl-tRNA Synthetase, Lead to Premature Ovarian Failure and Hearing Loss in Perrault Syndrome

(2013) Sarah B. Pierce et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Coronary heart disease is associated with a mutation in mitochondrial tRNA

(2013) Zidong Jia et al.   HUMAN MOLECULAR GENETICS

Phenotypic heterogeneity of the 8344A>G mtDNA "MERRF" mutation

(2013) M. Mancuso et al.   NEUROLOGY

Transfer RNA: A dancer between charging and mis-charging for protein biosynthesis

(2013) Zhou XiaoLong et al.   Science China-Life Sciences

Mitochondrial Stress Engages E2F1 Apoptotic Signaling to Cause Deafness

(2012) Nuno Raimundo et al.   CELL

Novel mitochondrial DNA mutations responsible for maternally inherited nonsyndromic hearing loss

(2012) Nicolás Gutiérrez Cortés et al.   HUMAN MUTATION

Mitochondrial tRNA mutations associated with deafness

(2012) Jing Zheng et al.   MITOCHONDRION

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

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

Human diseases with impaired mitochondrial protein synthesis

(2011) Agnès Rötig   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Assessing bioenergetic function in response to oxidative stress by metabolic profiling

(2011) Brian P. Dranka et al.   FREE RADICAL BIOLOGY AND MEDICINE

Nucleotide modifications and tRNA anticodon-mRNA codon interactions on the ribosome

(2011) O. Allner et al.   RNA

The Mitochondrial Proteome and Human Disease

(2010) Sarah E. Calvo et al.   Annual Review of Genomics and Human Genetics

Mitochondrial 12S rRNA mutations associated with aminoglycoside ototoxicity

(2010) Min-Xin Guan   MITOCHONDRION

Human Mitochondrial Leucyl-tRNA Synthetase Corrects Mitochondrial Dysfunctions Due to the tRNALeu(UUR) A3243G Mutation, Associated with Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-Like Symptoms and Diabetes

(2010) R. Li et al.   MOLECULAR AND CELLULAR BIOLOGY

Interaction of Aminoglycosides with Human Mitochondrial 12S rRNA Carrying the Deafness-Associated Mutation

(2009) Y. Qian et al.   ANTIMICROBIAL AGENTS AND CHEMOTHERAPY

Mitochondrial Transfer RNA Met 4435A>G Mutation Is Associated With Maternally Inherited Hypertension in a Chinese Pedigree

(2009) Yuqi Liu et al.   HYPERTENSION

Tertiary network in mammalian mitochondrial tRNAAsp revealed by solution probing and phylogeny

(2009) Marie Messmer et al.   NUCLEIC ACIDS RESEARCH

Evaluation of chemiluminescence and flow cytometry as tools in assessing production of hydrogen peroxide and superoxide anion in human spermatozoa

(2008) Reda Mahfouz et al.   FERTILITY AND STERILITY

A Disease-causing Point Mutation in Human Mitochondrial tRNAMetResults in tRNA Misfolding Leading to Defects in Translational Initiation and Elongation

(2008) Christie N. Jones et al.   JOURNAL OF BIOLOGICAL CHEMISTRY