Aminoacyl-tRNA synthetases

MAR Gomez, M Ibba - Rna, 2020 - rnajournal.cshlp.org
The aminoacyl-tRNA synthetases are an essential and universally distributed family of
enzymes that plays a critical role in protein synthesis, pairing tRNAs with their cognate …

Emerging roles of tRNA in adaptive translation, signalling dynamics and disease

S Kirchner, Z Ignatova - Nature Reviews Genetics, 2015 - nature.com
Abstract tRNAs, nexus molecules between mRNAs and proteins, have a central role in
translation. Recent discoveries have revealed unprecedented complexity of tRNA …

Translational fidelity and mistranslation in the cellular response to stress

K Mohler, M Ibba - Nature microbiology, 2017 - nature.com
Faithful translation of mRNA into the corresponding polypeptide is a complex multistep
process, requiring accurate amino acid selection, transfer RNA (tRNA) charging and mRNA …

Transfer RNA and human disease

JA Abbott, CS Francklyn, SM Robey-Bond - Frontiers in genetics, 2014 - frontiersin.org
Pathological mutations in tRNA genes and tRNA processing enzymes are numerous and
result in very complicated clinical phenotypes. Mitochondrial tRNA (mt-tRNA) genes are …

Exome sequencing identifies mitochondrial alanyl-tRNA synthetase mutations in infantile mitochondrial cardiomyopathy

A Götz, H Tyynismaa, L Euro, P Ellonen… - The American Journal of …, 2011 - cell.com
Infantile cardiomyopathies are devastating fatal disorders of the neonatal period or the first
year of life. Mitochondrial dysfunction is a common cause of this group of diseases, but the …

Synthetic and editing mechanisms of aminoacyl-tRNA synthetases

JJ Perona, I Gruic-Sovulj - Aminoacyl-tRNA synthetases in biology and …, 2014 - Springer
Aminoacyl-tRNA synthetases (aaRS) ensure the faithful transmission of genetic information
in all living cells. The 24 known aaRS families are divided into 2 structurally distinct classes …

Mitochondrial aminoacyl-tRNA synthetases in human disease

S Konovalova, H Tyynismaa - Molecular Genetics and Metabolism, 2013 - Elsevier
Mitochondrial aminoacyl-tRNA synthetases (mtARSs) are essential in the process of
transferring genetic information from mitochondrial DNA to the complexes of the oxidative …

Cellular mechanisms that control mistranslation

NM Reynolds, BA Lazazzera, M Ibba - Nature Reviews Microbiology, 2010 - nature.com
Mistranslation broadly encompasses the introduction of errors during any step of protein
synthesis, leading to the incorporation of an amino acid that is different from the one …

Quality control in aminoacyl-tRNA synthesis: its role in translational fidelity

SS Yadavalli, M Ibba - Advances in protein chemistry and structural biology, 2012 - Elsevier
Accurate translation of mRNA into protein is vital for maintenance of cellular integrity.
Translational fidelity is achieved by two key events: synthesis of correctly paired aminoacyl …

Loss-of-function alanyl-tRNA synthetase mutations cause an autosomal-recessive early-onset epileptic encephalopathy with persistent myelination defect

C Simons, LB Griffin, G Helman, G Golas… - The American Journal of …, 2015 - cell.com
Mutations in genes encoding aminoacyl-tRNA synthetases are known to cause
leukodystrophies and genetic leukoencephalopathies—heritable disorders that result in …