Published in Biochemistry on March 29, 2005
Deleterious mutation in the mitochondrial arginyl-transfer RNA synthetase gene is associated with pontocerebellar hypoplasia. Am J Hum Genet (2007) 3.55
Mutation of the mitochondrial tyrosyl-tRNA synthetase gene, YARS2, causes myopathy, lactic acidosis, and sideroblastic anemia--MLASA syndrome. Am J Hum Genet (2010) 2.07
Mitochondrial translation and beyond: processes implicated in combined oxidative phosphorylation deficiencies. J Biomed Biotechnol (2010) 2.01
Import of tRNAs and aminoacyl-tRNA synthetases into mitochondria. Curr Genet (2008) 1.31
Overexpression of human mitochondrial valyl tRNA synthetase can partially restore levels of cognate mt-tRNAVal carrying the pathogenic C25U mutation. Nucleic Acids Res (2008) 1.11
Biogenesis of glutaminyl-mt tRNAGln in human mitochondria. Proc Natl Acad Sci U S A (2009) 1.06
Dual-targeted tRNA-dependent amidotransferase ensures both mitochondrial and chloroplastic Gln-tRNAGln synthesis in plants. Proc Natl Acad Sci U S A (2008) 1.01
Mutation of the human mitochondrial phenylalanine-tRNA synthetase causes infantile-onset epilepsy and cytochrome c oxidase deficiency. Biochim Biophys Acta (2013) 0.97
A novel homozygous YARS2 mutation causes severe myopathy, lactic acidosis, and sideroblastic anemia 2. J Hum Genet (2014) 0.94
Phenotypic variability and identification of novel YARS2 mutations in YARS2 mitochondrial myopathy, lactic acidosis and sideroblastic anaemia. Orphanet J Rare Dis (2013) 0.92
Human mitochondrial TyrRS disobeys the tyrosine identity rules. RNA (2005) 0.92
Hepatitis B virus and Homo sapiens proteome-wide analysis: A profusion of viral peptide overlaps in neuron-specific human proteins. Biologics (2010) 0.92
Pleiotropic effects of a mitochondrial-nuclear incompatibility depend upon the accelerating effect of temperature in Drosophila. Genetics (2013) 0.89
The mitochondrial pool of free amino acids reflects the composition of mitochondrial DNA-encoded proteins: indication of a post- translational quality control for protein synthesis. Biosci Rep (2008) 0.89
A mechanism for functional segregation of mitochondrial and cytosolic genetic codes. Proc Natl Acad Sci U S A (2009) 0.86
tRNA biology in mitochondria. Int J Mol Sci (2015) 0.84
Thermodynamic properties distinguish human mitochondrial aspartyl-tRNA synthetase from bacterial homolog with same 3D architecture. Nucleic Acids Res (2012) 0.83
Viral hijacking of mitochondrial lysyl-tRNA synthetase. J Virol (2006) 0.83
The Roles of Compensatory Evolution and Constraint in Aminoacyl tRNA Synthetase Evolution. Mol Biol Evol (2015) 0.81
Tertiary network in mammalian mitochondrial tRNAAsp revealed by solution probing and phylogeny. Nucleic Acids Res (2009) 0.81
Decreased aminoacylation in pathology-related mutants of mitochondrial tRNATyr is associated with structural perturbations in tRNA architecture. RNA (2008) 0.80
Tyrosyl-tRNA synthetase: the first crystallization of a human mitochondrial aminoacyl-tRNA synthetase. Acta Crystallogr Sect F Struct Biol Cryst Commun (2007) 0.79
Crystal structure of Saccharomyces cerevisiae mitochondrial GatFAB reveals a novel subunit assembly in tRNA-dependent amidotransferases. Nucleic Acids Res (2014) 0.78
A Human Disease-causing Point Mutation in Mitochondrial Threonyl-tRNA Synthetase Induces Both Structural and Functional Defects. J Biol Chem (2016) 0.77
Evolutionary and structural annotation of disease-associated mutations in human aminoacyl-tRNA synthetases. BMC Genomics (2014) 0.76
Pathology-related mutation A7526G (A9G) helps in the understanding of the 3D structural core of human mitochondrial tRNA(Asp). RNA (2009) 0.76
Human Cytomegalovirus Infection Upregulates the Mitochondrial Transcription and Translation Machineries. MBio (2016) 0.76
A framework for application of metabolic modeling in yeast to predict the effects of nsSNV in human orthologs. Biol Direct (2014) 0.76
A novel therapeutic target for peripheral nerve injury-related diseases: aminoacyl-tRNA synthetases. Neural Regen Res (2015) 0.75
Hypobaric hypoxia-mediated protein expression in plasma of susceptible & tolerant rats. Indian J Med Res (2014) 0.75
Mitochondrial aspartyl-tRNA synthetase deficiency causes leukoencephalopathy with brain stem and spinal cord involvement and lactate elevation. Nat Genet (2007) 3.43
MITOS: improved de novo metazoan mitochondrial genome annotation. Mol Phylogenet Evol (2012) 3.41
Mutation of the mitochondrial tyrosyl-tRNA synthetase gene, YARS2, causes myopathy, lactic acidosis, and sideroblastic anemia--MLASA syndrome. Am J Hum Genet (2010) 2.07
Mamit-tRNA, a database of mammalian mitochondrial tRNA primary and secondary structures. RNA (2007) 1.72
Mitochondrial tRNA 3' end metabolism and human disease. Nucleic Acids Res (2004) 1.37
Kaposi's sarcoma herpesvirus microRNAs target caspase 3 and regulate apoptosis. PLoS Pathog (2011) 1.33
Microfluidic chips for the crystallization of biomacromolecules by counter-diffusion and on-chip crystal X-ray analysis. Lab Chip (2009) 1.29
Improved systematic tRNA gene annotation allows new insights into the evolution of mitochondrial tRNA structures and into the mechanisms of mitochondrial genome rearrangements. Nucleic Acids Res (2011) 1.22
Towards understanding human mitochondrial leucine aminoacylation identity. J Mol Biol (2003) 1.19
Structure of transfer RNAs: similarity and variability. Wiley Interdiscip Rev RNA (2011) 1.18
A pathogenesis-associated mutation in human mitochondrial tRNALeu(UUR) leads to reduced 3'-end processing and CCA addition. J Mol Biol (2004) 1.14
Analysis of small RNA in fission yeast; centromeric siRNAs are potentially generated through a structured RNA. EMBO J (2009) 1.14
Virus-encoded aminoacyl-tRNA synthetases: structural and functional characterization of mimivirus TyrRS and MetRS. J Virol (2007) 1.13
Atypical archaeal tRNA pyrrolysine transcript behaves towards EF-Tu as a typical elongator tRNA. Nucleic Acids Res (2004) 1.11
Physical aspects of protein crystal growth investigated with the Advanced Protein Crystallization Facility in reduced-gravity environments. Acta Crystallogr D Biol Crystallogr (2002) 1.10
Crystal structure of human mitochondrial tyrosyl-tRNA synthetase reveals common and idiosyncratic features. Structure (2007) 1.07
An aminoacyl-tRNA synthetase-like protein encoded by the Escherichia coli yadB gene glutamylates specifically tRNAAsp. Proc Natl Acad Sci U S A (2004) 1.06
Non-discriminating and discriminating aspartyl-tRNA synthetases differ in the anticodon-binding domain. EMBO J (2003) 1.05
Expression of metazoan replication-dependent histone genes. Biochimie (2005) 1.05
The Escherichia coli YadB gene product reveals a novel aminoacyl-tRNA synthetase like activity. J Mol Biol (2004) 1.05
Crystal growth of proteins, nucleic acids, and viruses in gels. Prog Biophys Mol Biol (2009) 1.04
Evolution of the tRNA(Tyr)/TyrRS aminoacylation systems. Biochimie (2005) 1.04
Recognition of human mitochondrial tRNALeu(UUR) by its cognate leucyl-tRNA synthetase. J Mol Biol (2004) 0.99
tRNAs and tRNA mimics as cornerstones of aminoacyl-tRNA synthetase regulations. Biochimie (2005) 0.99
Yeast aspartyl-tRNA synthetase binds specifically its own mRNA. J Mol Biol (2003) 0.98
Glu-Q-tRNA(Asp) synthetase coded by the yadB gene, a new paralog of aminoacyl-tRNA synthetase that glutamylates tRNA(Asp) anticodon. Biochimie (2005) 0.95
Deinococcus glutaminyl-tRNA synthetase is a chimer between proteins from an ancient and the modern pathways of aminoacyl-tRNA formation. Nucleic Acids Res (2007) 0.94
Pathology-related substitutions in human mitochondrial tRNA(Ile) reduce precursor 3' end processing efficiency in vitro. Nucleic Acids Res (2003) 0.94
Towards atomic resolution with crystals grown in gel: the case of thaumatin seen at room temperature. Proteins (2002) 0.94
tRNA-like structure regulates translation of Brome mosaic virus RNA. J Virol (2004) 0.94
A minimalist glutamyl-tRNA synthetase dedicated to aminoacylation of the tRNAAsp QUC anticodon. Nucleic Acids Res (2004) 0.93
Human mitochondrial TyrRS disobeys the tyrosine identity rules. RNA (2005) 0.92
Phenotypic variability and identification of novel YARS2 mutations in YARS2 mitochondrial myopathy, lactic acidosis and sideroblastic anaemia. Orphanet J Rare Dis (2013) 0.92
Crystallization in the presence of glycerol displaces water molecules in the structure of thaumatin. Acta Crystallogr D Biol Crystallogr (2002) 0.89
Evidence for the existence in mRNAs of a hairpin element responsible for ribosome dependent pyrrolysine insertion into proteins. Biochimie (2005) 0.89
Activation of the hetero-octameric ATP phosphoribosyl transferase through subunit interface rearrangement by a tRNA synthetase paralog. J Biol Chem (2005) 0.89
Comparative analysis of space-grown and earth-grown crystals of an aminoacyl-tRNA synthetase: space-grown crystals are more useful for structural determination. Acta Crystallogr D Biol Crystallogr (2002) 0.89
Lessons from crystals grown in the Advanced Protein Crystallisation Facility for conventional crystallisation applied to structural biology. Biophys Chem (2005) 0.89
Low Complexity Regions behave as tRNA sponges to help co-translational folding of plasmodial proteins. FEBS Lett (2010) 0.88
Aminoacylation properties of pathology-related human mitochondrial tRNA(Lys) variants. RNA (2004) 0.88
tRNA-balanced expression of a eukaryal aminoacyl-tRNA synthetase by an mRNA-mediated pathway. EMBO Rep (2005) 0.88
Binding of tobramycin leads to conformational changes in yeast tRNA(Asp) and inhibition of aminoacylation. EMBO J (2002) 0.87
Structure of thaumatin in a hexagonal space group: comparison of packing contacts in four crystal lattices. Acta Crystallogr D Biol Crystallogr (2003) 0.87
Crystal contacts engineering of aspartyl-tRNA synthetase from Thermus thermophilus: effects on crystallizability. Acta Crystallogr D Biol Crystallogr (2002) 0.87
Prevalence of rare mitochondrial DNA mutations in mitochondrial disorders. J Med Genet (2013) 0.87
Armless mitochondrial tRNAs in Enoplea (Nematoda). RNA Biol (2012) 0.87
Yeast tRNA(Asp) charging accuracy is threatened by the N-terminal extension of aspartyl-tRNA synthetase. J Biol Chem (2002) 0.86
A human pathology-related mutation prevents import of an aminoacyl-tRNA synthetase into mitochondria. Biochem J (2011) 0.86
Binding of human SLBP on the 3'-UTR of histone precursor H4-12 mRNA induces structural rearrangements that enable U7 snRNA anchoring. Nucleic Acids Res (2006) 0.85
Transfer RNA recognition by class I lysyl-tRNA synthetase from the Lyme disease pathogen Borrelia burgdorferi. FEBS Lett (2005) 0.85
Functional idiosyncrasies of tRNA isoacceptors in cognate and noncognate aminoacylation systems. Biochimie (2004) 0.84
Pathogenic implications of human mitochondrial aminoacyl-tRNA synthetases. Top Curr Chem (2014) 0.83
Thermodynamic properties distinguish human mitochondrial aspartyl-tRNA synthetase from bacterial homolog with same 3D architecture. Nucleic Acids Res (2012) 0.83
Peculiar inhibition of human mitochondrial aspartyl-tRNA synthetase by adenylate analogs. Biochimie (2009) 0.83
Pathogenic mutations causing LBSL affect mitochondrial aspartyl-tRNA synthetase in diverse ways. Biochem J (2013) 0.82
Adaptation of aminoacylation identity rules to mammalian mitochondria. Biochimie (2012) 0.82
Comparative proteomics as a new tool for exploring human mitochondrial tRNA disorders. Biochemistry (2002) 0.82
Genetic code expansion. Nat Struct Biol (2003) 0.82
An intricate RNA structure with two tRNA-derived motifs directs complex formation between yeast aspartyl-tRNA synthetase and its mRNA. J Mol Biol (2005) 0.82
Idiosyncratic behaviour of tRNA-like structures in translation of plant viral RNA genomes. J Mol Biol (2005) 0.82
Tertiary network in mammalian mitochondrial tRNAAsp revealed by solution probing and phylogeny. Nucleic Acids Res (2009) 0.81
Handling mammalian mitochondrial tRNAs and aminoacyl-tRNA synthetases for functional and structural characterization. Methods (2008) 0.81
A yeast arginine specific tRNA is a remnant aspartate acceptor. Nucleic Acids Res (2004) 0.81
Decreased aminoacylation in pathology-related mutants of mitochondrial tRNATyr is associated with structural perturbations in tRNA architecture. RNA (2008) 0.80
Tyrosyl-tRNA synthetase: the first crystallization of a human mitochondrial aminoacyl-tRNA synthetase. Acta Crystallogr Sect F Struct Biol Cryst Commun (2007) 0.79
Loss of a primordial identity element for a mammalian mitochondrial aminoacylation system. J Biol Chem (2006) 0.79
Functionalized self-assembled monolayer on gold for detection of human mitochondrial tRNA gene mutations. Anal Biochem (2003) 0.78