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A mutation in the tRNALeu(UUR) gene associated with the MELAS subgroup of mitochondrial encephalomyopathies

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Myoclonic epilepsy and ragged-red fiber disease (MERRF) is associated with a mitochondrial DNA tRNALys mutation

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MELAS mutation in mtDNA binding site for transcription termination factor causes defects in protein synthesis and in respiration but no change in levels of upstream and downstream mature transcripts.

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A new mtDNA mutation associated with mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS)

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Phy M: an RNase activity specific for U and A residues useful in RNA sequence analysis

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In Vitro Genetic Transfer of Protein Synthesis and Respiration Defects to Mitochondrial DNA-Less Cells with Myopathy-Patient Mitochondria

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MtDNA mutation in MERRF syndrome causes defective aminoacylation of tRNALys and premature translation termination

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Different pattern of codon recognition by mammalian mitochondrial tRNAs.

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Novel features in the genetic code and codon reading patterns in Neurospora crassa mitochondria based on sequences of six mitochondrial tRNAs

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The presence of modified nucleotides is required for cloverleaf folding of a human mitochondrial tRNA

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Complementation of Mutant and Wild-Type Human Mitochondrial DNAs Coexisting Since the Mutation Event and Lack of Complementation of DNAs Introduced Separately into a Cell within Distinct Organelles

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Mobilities of modified ribonucleotides on two-dimensional cellulose thin-layer chromatography

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The ‘polysemous’ codon—a codon with multiple amino acid assignment caused by dual specificity of tRNA identity

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Respiration-deficient cells are caused by a single point mutation in the mitochondrial tRNA-Leu (UUR) gene in mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS).

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The Diabetes-associated 3243 Mutation in the Mitochondrial tRNALeu(UUR) Gene Causes Severe Mitochondrial Dysfunction without a Strong Decrease in Protein Synthesis Rate

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Higher-order structure of bovine mitochondrial tRNAPhelacking the ‘conserved’ GG and TΨCG sequences as inferred by enzymatic and chemical probing

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[24] Analysis of modified nucleosides and nucleotide sequence of tRNA

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Accumulation of mtDNA with a Mutation at Position 3271 in tRNALeu(UUR) Gene Introduced from a Melas Patient to HeLa Cells Lacking mtDNA Results in Progressive Inhibition of Mitochondrial Respiratory Function

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A Novel Wobble Rule Found in Starfish Mitochondria

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Translation of Synonymous Codons in Family Boxes byMycoplasma capricolumtRNAs with Unmodified Uridine or Adenosine at the First Anticodon Position

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7-Methylguanosine at the anticodon wobble position of squid mitochondrial tRNASerGCU: molecular basis for assignment of AGA/AGG codons as serine in invertebrate mitochondria

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Unconventional reading of the glycine codons.

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High Sensitive Analysis of Modified Nucleosides by LC/MS Using ESI/Iontrap Mass Spectrometry.

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The expanding world of tRNA modifications and their disease relevance

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Mitochondrial DNA mutations and human disease

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

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Role of tRNA modifications in human diseases

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Taurine as a constituent of mitochondrial tRNAs: new insights into the functions of taurine and human mitochondrial diseases

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Translational down-regulation of gene expression

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Wobble modification defect in tRNA disturbs codon–anticodon interaction in a mitochondrial disease

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Wobble modification defect suppresses translational activity of tRNAs with MERRF and MELAS mutations

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P472 The role of oculographic recordings in mitochondrial encephalomyopathies

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P473 Saccadic eye movements during intracarotid amobarbital anesthesia

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