Human mitochondrial tRNA quality control in health and disease

Mutations in the Mitochondrial Seryl-tRNA Synthetase Cause Hyperuricemia, Pulmonary Hypertension, Renal Failure in Infancy and Alkalosis, HUPRA Syndrome

(2011) Ruth Belostotsky et al.   AMERICAN JOURNAL OF HUMAN GENETICS

tRNA Stabilization by Modified Nucleotides

(2010) Yuri Motorin et al.   BIOCHEMISTRY

PNPASE Regulates RNA Import into Mitochondria

(2010) Geng Wang et al.   CELL

tRNA biology charges to the front

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

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

Scyl1 Facilitates Nuclear tRNA Export in Mammalian Cells by Acting at the Nuclear Pore Complex

(2010) Shawn C. Chafe et al.   MOLECULAR BIOLOGY OF THE CELL

Deep sequencing of Salmonella RNA associated with heterologous Hfq proteins in vivo reveals small RNAs as a major target class and identifies RNA processing phenotypes

(2010) Alexandra Sittka et al.   RNA Biology

Acute Infantile Liver Failure Due to Mutations in the TRMU Gene

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

Processivity of translation in the eukaryote cell: Role of aminoacyl-tRNA synthetases

(2009) Marc Mirande   FEBS LETTERS

Can yeast be used to study mitochondrial diseases? Biolistic tRNA mutants for the analysis of mechanisms and suppressors

(2009) Cristina De Luca et al.   MITOCHONDRION

The Role of Aminoacyl-tRNA Synthetases in Genetic Diseases

(2008) Anthony Antonellis et al.   Annual Review of Genomics and Human Genetics

Degradation of several hypomodified mature tRNA species in Saccharomyces cerevisiae is mediated by Met22 and the 5'-3' exonucleases Rat1 and Xrn1

(2008) I. Chernyakov et al.   GENES & DEVELOPMENT

The A3243G tRNALeu(UUR) MELAS mutation causes amino acid misincorporation and a combined respiratory chain assembly defect partially suppressed by overexpression of EFTu and EFG2

(2008) F. Sasarman et al.   HUMAN MOLECULAR GENETICS

Overexpression of human mitochondrial valyl tRNA synthetase can partially restore levels of cognate mt-tRNAVal carrying the pathogenic C25U mutation

(2008) Joanna Rorbach et al.   NUCLEIC ACIDS RESEARCH

Overexpressed mitochondrial leucyl-tRNA synthetase suppresses the A3243G mutation in the mitochondrial tRNALeu(UUR) gene

(2008) H. Park et al.   RNA