Gene Symbol: AARS
Description: alanyl-tRNA synthetase
Alias: CMT2N, EIEE29, alanine--tRNA ligase, cytoplasmic, alaRS, alanine tRNA ligase 1, cytoplasmic, alanyl-tRNA synthetase, cytoplasmic, renal carcinoma antigen NY-REN-42
Species: human
Products:     AARS

Top Publications

  1. Dulic M, Perona J, Gruic Sovulj I. Determinants for tRNA-dependent pretransfer editing in the synthetic site of isoleucyl-tRNA synthetase. Biochemistry. 2014;53:6189-98 pubmed publisher
    ..expression of genetic information relies on the fidelity of amino acid-tRNA coupling by aminoacyl-tRNA synthetases (aaRS)...
  2. Willis J, Chin J. Mutually orthogonal pyrrolysyl-tRNA synthetase/tRNA pairs. Nat Chem. 2018;10:831-837 pubmed publisher
    ..amino acids (ncAAs) into proteins synthesized in cells requires mutually orthogonal aminoacyl-tRNA synthetase (aaRS)/tRNA pairs...
  3. Ji Q, Fang Z, Ye Q, Chi C, Wang E. Self-protective responses to norvaline-induced stress in a leucyl-tRNA synthetase editing-deficient yeast strain. Nucleic Acids Res. 2017;45:7367-7381 pubmed publisher
    ..In conclusion, our study highlights the significance of the editing function of LeuRS and provides clues for understanding the intracellular stress protective mechanisms that are triggered in aaRS editing-deficient organisms.
  4. Sharma A, Sharma A. Plasmodium falciparum mitochondria import tRNAs along with an active phenylalanyl-tRNA synthetase. Biochem J. 2015;465:459-69 pubmed publisher
    ..The aaRS ensemble catalyses transfer of amino acids to cognate tRNAs, thus providing charged tRNAs for ribosomal consumption...
  5. Yin X, Tang B, Mao X, Peng J, Zeng S, Wang Y, et al. The genotypic and phenotypic spectrum of PARS2-related infantile-onset encephalopathy. J Hum Genet. 2018;: pubmed publisher
    ..Mutations in mt-aaRSs are associated with various diseases. As a member of the mt-aaRS family, PARS2 encoding prolyl-tRNA synthetase 2 was recently shown to be associated with Alpers syndrome and ..
  6. Vo M, Terrey M, Lee J, Roy B, Moresco J, Sun L, et al. ANKRD16 prevents neuron loss caused by an editing-defective tRNA synthetase. Nature. 2018;557:510-515 pubmed publisher
    ..A mutation in the editing domain of alanyl tRNA synthetase (AlaRS) in Aars sti mutant mice results in an increase in the production of serine-mischarged tRNAAla
  7. Schmidt M, Summerer D. Directed Evolution of Orthogonal Pyrrolysyl-tRNA Synthetases in Escherichia coli for the Genetic Encoding of Noncanonical Amino Acids. Methods Mol Biol. 2018;1728:97-111 pubmed publisher
    The directed evolution of orthogonal aminoacyl-tRNA synthetases (aaRS) for the genetic encoding of noncanonical amino acids (ncAA) has paved the way for the site-specific incorporation of >170 functionally diverse ncAAs into proteins ..
  8. Reichert A, Poxleitner G, Dauner M, Skerra A. Optimisation of a system for the co-translational incorporation of a keto amino acid and its application to a tumour-specific Anticalin. Protein Eng Des Sel. 2015;28:553-65 pubmed publisher
    ..e. the modified aminoacyl-tRNA synthetase (aaRS), the cognate amber suppressor tRNA and the enhanced green fluorescent protein equipped with an amber stop codon ..
  9. Park B, Jo H, Jung J. Expression profile of aminoacyl-tRNA synthetases in dorsal root ganglion neurons after peripheral nerve injury. J Mol Histol. 2015;46:115-22 pubmed publisher
    ..Here, to explore the cellular changes in sensory neurons after nerve injury, we evaluated AARS mRNA expression in rat dorsal root ganglia (DRG) neurons using AminoARS-specific primers...

More Information


  1. Lu J, Bergert M, Walther A, Suter B. Double-sieving-defective aminoacyl-tRNA synthetase causes protein mistranslation and affects cellular physiology and development. Nat Commun. 2014;5:5650 pubmed publisher
    ..Recently, aaRS mutations have been linked to various human diseases; however, how these mutations lead to diseases has remained ..
  2. Di Micco P, Fazzi D Orsi M, Morea V, Frontali L, Francisci S, Montanari A. The yeast model suggests the use of short peptides derived from mt LeuRS for the therapy of diseases due to mutations in several mt tRNAs. Biochim Biophys Acta. 2014;1843:3065-74 pubmed publisher
    ..be relieved by overexpression of both cognate and non-cognate nuclearly encoded mt aminoacyl-tRNA synthetases (aaRS) LeuRS, IleRS and ValRS...
  3. Fuchs S, Schene I, Kok G, Jansen J, Nikkels P, van Gassen K, et al. Aminoacyl-tRNA synthetase deficiencies in search of common themes. Genet Med. 2018;: pubmed publisher
    ..In literature, we identified 107 patients with AARS, DARS, GARS, HARS, IARS, KARS, LARS, MARS, RARS, SARS, VARS, YARS, and QARS deficiencies...
  4. Debard S, Bader G, De Craene J, Enkler L, Bär S, Laporte D, et al. Nonconventional localizations of cytosolic aminoacyl-tRNA synthetases in yeast and human cells. Methods. 2017;113:91-104 pubmed publisher
    ..other translationally-active compartments like mitochondria and plastids can simultaneously contain the cytosolic aaRS and its corresponding organellar ortholog suggesting that both forms do not share the same organellar function...
  5. Farazi P, Watanabe Galloway S, Westman L, Rettig B, Hunt P, Cammack R, et al. Temporal and geospatial trends of pediatric cancer incidence in Nebraska over a 24-year period. Cancer Epidemiol. 2018;52:83-90 pubmed publisher
    ..SEER AARs were also calculated as a proxy for pediatric cancer incidence in the United States (1990-2013) and compared to the ..
  6. Shi Y, Wei N, Yang X. Studying nuclear functions of aminoacyl tRNA synthetases. Methods. 2017;113:105-110 pubmed publisher
    ..Application of these methods would expand our understanding of AARS functions and reveal critical insights on the coordination of gene transcription and translation.
  7. Sissler M, González Serrano L, Westhof E. Recent Advances in Mitochondrial Aminoacyl-tRNA Synthetases and Disease. Trends Mol Med. 2017;23:693-708 pubmed publisher
    ..With newer information available, we propose that the pleiotropy and tissue-specificity of mt-aaRS-associated diseases result from the molecular integration of mitochondrial translation events within the cell; ..
  8. Escolar Pujolar A, Córdoba Doña J, Goicolea Julían I, Rodríguez G, Santos Sánchez V, Mayoral Sánchez E, et al. The effect of marital status on social and gender inequalities in diabetes mortality in Andalusia. Endocrinol Diabetes Nutr. 2018;65:21-29 pubmed publisher
    ..DM deaths between 2002 and 2013 were analyzed by educational level and marital status. Age-adjusted rates (AARs) and mortality rate ratios (MRRs) were calculated using Poisson regression models, controlling for several social ..
  9. Randall C, Rasina D, Jirgensons A, O Neill A. Targeting Multiple Aminoacyl-tRNA Synthetases Overcomes the Resistance Liabilities Associated with Antibacterial Inhibitors Acting on a Single Such Enzyme. Antimicrob Agents Chemother. 2016;60:6359-61 pubmed publisher
    Bacterial aminoacyl-tRNA synthetases (aaRSs) represent promising antibacterial drug targets. Unfortunately, the aaRS inhibitors that have to date reached clinical trials are subject to rapid resistance development through mutation, a ..
  10. Mirando A, Fang P, Williams T, Baldor L, Howe A, Ebert A, et al. Aminoacyl-tRNA synthetase dependent angiogenesis revealed by a bioengineered macrolide inhibitor. Sci Rep. 2015;5:13160 pubmed publisher
    ..These include angiogenesis, and human threonyl-tRNA synthetase (TARS) represents a potent pro-angiogenic AARS. Angiogenesis stimulation can be blocked by the macrolide antibiotic borrelidin (BN), which exhibits a broad ..
  11. Ren W, Truong T, Ai H. Study of the Binding Energies between Unnatural Amino Acids and Engineered Orthogonal Tyrosyl-tRNA Synthetases. Sci Rep. 2015;5:12632 pubmed publisher
    ..and ROSETTA were able to distinguish binding energy differences for individual pairs of favorable and unfavorable aaRS-amino acid complexes, but were unable to cluster together all experimentally verified favorable complexes from ..
  12. Zhang B, De Graef S, Nautiyal M, Pang L, Gadakh B, Froeyen M, et al. Family-wide analysis of aminoacyl-sulfamoyl-3-deazaadenosine analogues as inhibitors of aminoacyl-tRNA synthetases. Eur J Med Chem. 2018;148:384-396 pubmed publisher
    ..Aminoacyl-sulfamoyl adenosines (aaSAs) are potent orthologue specific aaRS inhibitors that demonstrate nanomolar affinities in vitro but have limited uptake...
  13. Kinon M, Nasser R, Nakhla J, Desai R, Moreno J, Yassari R, et al. Atlantoaxial Rotatory Subluxation: A Review for the Pediatric Emergency Physician. Pediatr Emerg Care. 2016;32:710-716 pubmed
    Pediatric emergency physicians must have a high clinical suspicion for atlantoaxial rotatory subluxation (AARS), particularly when a child presents with neck pain and an abnormal head posture without the ability to return to a neutral ..
  14. Stephen P, Lin S, Giege R. Interplay between Catalysts and Substrates for Activity of Class Ib Aminoacyl-tRNA Synthetases and Implications for Pharmacology. Curr Top Med Chem. 2016;16:616-33 pubmed
    Aminoacyl-tRNA synthetase:transfer RNA (aaRS:tRNA) systems became recently essential targets in molecular medicine, because perturbed recognition of cognate tRNAs by aaRSs and poor precision in tRNA aminoacylation do not guarantee ..
  15. Eghbal K, Derakhshan N, Haghighat A. Ocular Manifestation of a Cervical Spine Injury: An Adult Case of Traumatic Atlantoaxial Rotatory Subluxation Manifesting with Nystagmus. World Neurosurg. 2017;101:817.e1-817.e3 pubmed publisher
    Atlantoaxial rotatory subluxation (AARS) is a rare type of traumatic cervical spine injury in adults, commonly manifesting with painful torticollis and suboccipital headache. Early diagnosis is mandatory to avoid catastrophic consequences...
  16. Hu J, Tian J, Li F, Xue B, Hu J, Cheng X, et al. Clone and functional analysis of Seryl-tRNA synthetase and Tyrosyl-tRNA synthetase from silkworm, Bombyx mori. Sci Rep. 2017;7:41563 pubmed publisher
    ..Among them, the genes of alanyl-tRNA synthetase (AlaRS) and glycyl-tRNA synthetase (GlyRS) have been cloned...
  17. Amiram M, Haimovich A, Fan C, Wang Y, Aerni H, Ntai I, et al. Evolution of translation machinery in recoded bacteria enables multi-site incorporation of nonstandard amino acids. Nat Biotechnol. 2015;33:1272-1279 pubmed publisher
    ..This approach to aaRS evolution should accelerate and expand our ability to produce functionalized proteins and sequence-defined polymers ..
  18. Wang Y, Zhou X, Ruan Z, Liu R, Eriani G, Wang E. A Human Disease-causing Point Mutation in Mitochondrial Threonyl-tRNA Synthetase Induces Both Structural and Functional Defects. J Biol Chem. 2016;291:6507-20 pubmed publisher
    ..Some aaRS mutations cause mitochondrial disorders, including human mitochondrial threonyl-tRNA synthetase (hmtThrRS) (encoded ..
  19. Chakraborty S, Mazumder T, Uddin A. Compositional dynamics and codon usage pattern of BRCA1 gene across nine mammalian species. Genomics. 2018;: pubmed publisher
    ..31 and plays important role in biological processes. The aminoacyl-tRNA synthetases (AARS) are a family of heterogenous enzymes responsible protein synthesis and whose secondary functions include a role in ..
  20. Fang P, Guo M. Structural characterization of human aminoacyl-tRNA synthetases for translational and nontranslational functions. Methods. 2017;113:83-90 pubmed publisher
    ..Structural characterization of aaRS functions in both categories has deepened our understanding and provided insightful platform for further structure-..
  21. Sapienza P, Li L, Williams T, Lee A, Carter C. An Ancestral Tryptophanyl-tRNA Synthetase Precursor Achieves High Catalytic Rate Enhancement without Ordered Ground-State Tertiary Structures. ACS Chem Biol. 2016;11:1661-8 pubmed publisher
    ..Urzymes-short, active core modules derived from enzyme superfamilies-prepared from the two aminoacyl-tRNA synthetase (aaRS) classes contain only the modules shared by all related family members...
  22. Ruhfel B, Bove C, Philbrick C, Davis C. Dispersal largely explains the Gondwanan distribution of the ancient tropical clusioid plant clade. Am J Bot. 2016;103:1117-28 pubmed publisher
    ..Ancestral Area Reconstructions (AARs) were then conducted on a distribution of 1000 trees and summarized on a reduced phylogeny...
  23. Chung Y. Seemingly irrational driving behavior model: The effect of habit strength and anticipated affective reactions. Accid Anal Prev. 2015;82:79-89 pubmed publisher
    ..Anticipated affective reactions (AARs) were further included to examine the effect of affect on aberrant driving behaviors...
  24. He J, MELANCON C. Genetic Incorporation of Unnatural Amino Acids into Proteins of Interest in Streptomyces venezuelae ATCC 15439. Methods Mol Biol. 2018;1728:155-168 pubmed publisher
    ..unnatural amino acids (UAAs) into proteins in living cells using engineered orthogonal aminoacyl-tRNA synthetase (aaRS)/tRNA pairs is a powerful tool for studying and manipulating protein structure and function...
  25. Mukai T, Reynolds N, Crnkovic A, Soll D. Bioinformatic Analysis Reveals Archaeal tRNATyr and tRNATrp Identities in Bacteria. Life (Basel). 2017;7: pubmed publisher
    ..HIGH-motif and KMSKS-motif as well as many ?-helices that are characteristic of class I aminoacyl-tRNA synthetase (aaRS) homologs...
  26. Adrion J, White P, Montooth K. The Roles of Compensatory Evolution and Constraint in Aminoacyl tRNA Synthetase Evolution. Mol Biol Evol. 2016;33:152-61 pubmed publisher
    ..RNAs encoded by the mitochondrial genome (mt-tRNAs) and mitochondrial aminoacyl tRNA synthetase proteins (mt-aaRS) encoded by the nuclear genome...
  27. Fang P, Guo M. Evolutionary Limitation and Opportunities for Developing tRNA Synthetase Inhibitors with 5-Binding-Mode Classification. Life (Basel). 2015;5:1703-25 pubmed publisher
    ..Each of the aaRS families plays a pivotal role in protein biosynthesis and is indispensable for cell growth and survival...
  28. Seki E, Yanagisawa T, Yokoyama S. Cell-Free Protein Synthesis for Multiple Site-Specific Incorporation of Noncanonical Amino Acids Using Cell Extracts from RF-1 Deletion E. coli Strains. Methods Mol Biol. 2018;1728:49-65 pubmed publisher
    ..A::Z and B-95.?A, with orthogonal tRNA and aminoacyl-tRNA synthetase (aaRS) pairs from Methanosarcina mazei, we have developed CFPS methods for the highly productive and efficient multiple ..
  29. Zhang J, Yan B, Späth S, Qun H, Cornelius S, Guan D, et al. Integrated transcriptional profiling and genomic analyses reveal RPN2 and HMGB1 as promising biomarkers in colorectal cancer. Cell Biosci. 2015;5:53 pubmed publisher
    ..This led to the identification of eight candidate genes including RPN2, HMGB1, AARS, IGFBP3, STAT1, HYOU1, NQO1 and PEA15 that were associated with the progressive phenotype...
  30. Cantara W, Olson E, Musier Forsyth K. Analysis of RNA structure using small-angle X-ray scattering. Methods. 2017;113:46-55 pubmed publisher
    In addition to their role in correctly attaching specific amino acids to cognate tRNAs, aminoacyl-tRNA synthetases (aaRS) have been found to possess many alternative functions and often bind to and act on other nucleic acids...
  31. Crnkovic A, Čavužić M, Godinic Mikulcic V, Anderluh G, Weygand Đurašević I, Gruic Sovulj I. An archaeal aminoacyl-tRNA synthetase complex for improved substrate quality control. Biochimie. 2018;147:36-45 pubmed publisher
    ..Propensity of the ArgRS•SerRS complex to exclude unmodified tRNAs from translation leads to an attractive hypothesis that SerRS•ArgRS complex might act in vivo as a safeguarding switch that improves translation accuracy. ..
  32. Holman K, Wu J, Ling J, Simonović M. The crystal structure of yeast mitochondrial ThrRS in complex with the canonical threonine tRNA. Nucleic Acids Res. 2016;44:1428-39 pubmed publisher
    In mitochondria of Saccharomyces cerevisiae, a single aminoacyl-tRNA synthetase (aaRS), MST1, aminoacylates two isoacceptor tRNAs, tRNA1(Thr) and tRNA2(Thr), that harbor anticodon loops of different size and sequence...
  33. Wiener H, Shrestha S, Lu H, Karita E, Kilembe W, Allen S, et al. Immunogenetic factors in early immune control of human immunodeficiency virus type 1 (HIV-1) infection: Evaluation of HLA class I amino acid variants in two African populations. Hum Immunol. 2018;79:166-171 pubmed publisher
    ..Recent work has uncovered specific amino acid residues (AARs) that seem to dictate the extent of immune control in African Americans, which prompted us to test these emerging ..
  34. Yang X, Xiong Y, Duan H, Gong R. Identification of genes associated with methotrexate resistance in methotrexate-resistant osteosarcoma cell lines. J Orthop Surg Res. 2015;10:136 pubmed publisher
    ..A total of 690 up-regulated genes and down-regulated 626 genes were identified. Up-regulated DEGs (including AARS and PARS2) were associated to transfer RNA (tRNA) aminoacylation while down-regulated DEGs (including AURKA, CCNB1, ..
  35. Martinez Rodriguez L, Erdogan O, Jimenez Rodriguez M, Gonzalez Rivera K, Williams T, Li L, et al. Functional Class I and II Amino Acid-activating Enzymes Can Be Coded by Opposite Strands of the Same Gene. J Biol Chem. 2015;290:19710-25 pubmed publisher
    Aminoacyl-tRNA synthetases (aaRS) catalyze both chemical steps that translate the universal genetic code...
  36. Dumas A, Lercher L, Spicer C, Davis B. Designing logical codon reassignment - Expanding the chemistry in biology. Chem Sci. 2015;6:50-69 pubmed publisher
    ..proteins relies on the reassignment or suppression of canonical codons with an amino-acyl tRNA synthetase/tRNA (aaRS/tRNA) pair, selective for the UAA of choice...
  37. Abbott J, Livingston N, Egri S, Guth E, Francklyn C. Characterization of aminoacyl-tRNA synthetase stability and substrate interaction by differential scanning fluorimetry. Methods. 2017;113:64-71 pubmed publisher
    ..Here, we describe the application of DSF to investigate aminoacyl-tRNA synthetase (AARS) stability and interaction with ligands...
  38. Song Y, Zhou H, Vo M, Shi Y, Nawaz M, Vargas Rodriguez O, et al. Double mimicry evades tRNA synthetase editing by toxic vegetable-sourced non-proteinogenic amino acid. Nat Commun. 2017;8:2281 pubmed publisher
    ..While aminoacyl-tRNA synthetase (AARS) editing potentially provides a mechanism to reject np AAs, some have pathological associations...
  39. Azuma Y, Zschoche R, Hilvert D. The C-terminal peptide of Aquifex aeolicus riboflavin synthase directs encapsulation of native and foreign guests by a cage-forming lumazine synthase. J Biol Chem. 2017;292:10321-10327 pubmed publisher
    ..i>Aquifex aeolicus (AaLS) and found that it also forms cage complexes with the cognate riboflavin synthase (AaRS) when both proteins are co-produced in the cytosol of Escherichia coli A 12-amino acid-long peptide at the C ..
  40. Kumar M, Kumar S, Dimkovikj A, Baykal L, Banton M, Outlaw M, et al. Zinc is the molecular "switch" that controls the catalytic cycle of bacterial leucyl-tRNA synthetase. J Inorg Biochem. 2015;142:59-67 pubmed publisher
    The Escherichia coli (E. coli) leucyl-tRNA synthetase (LeuRS) enzyme is part of the aminoacyl-tRNA synthetase (aaRS) family. LeuRS is an essential enzyme that relies on specialized domains to facilitate the aminoacylation reaction...
  41. Kubyshkin V, Acevedo Rocha C, Budisa N. On universal coding events in protein biogenesis. Biosystems. 2018;164:16-25 pubmed publisher
    ..It starts with amino acid activation by aminoacyl tRNA synthetases (aaRS) followed by matching with the acceptor units of their cognate tRNAs ("operational RNA code") and ribosomal codon-..
  42. Englert M, Vargas Rodriguez O, Reynolds N, Wang Y, Söll D, Umehara T. A genomically modified Escherichia coli strain carrying an orthogonal E. coli histidyl-tRNA synthetase•tRNAHis pair. Biochim Biophys Acta. 2017;1861:3009-3015 pubmed publisher
    Development of new aminoacyl-tRNA synthetase (aaRS)•tRNA pairs is central for incorporation of novel non-canonical amino acids (ncAAs) into proteins via genetic code expansion (GCE)...
  43. Götz A, Tyynismaa H, Euro L, Ellonen P, Hyötyläinen T, Ojala T, et al. Exome sequencing identifies mitochondrial alanyl-tRNA synthetase mutations in infantile mitochondrial cardiomyopathy. Am J Hum Genet. 2011;88:635-42 pubmed publisher
    ..Furthermore, we show that mitochondrial disorders extend to prenatal life and are an important cause of early infantile cardiac failure. ..
  44. McLaughlin H, Sakaguchi R, Giblin W, Wilson T, Biesecker L, Lupski J, et al. A recurrent loss-of-function alanyl-tRNA synthetase (AARS) mutation in patients with Charcot-Marie-Tooth disease type 2N (CMT2N). Hum Mutat. 2012;33:244-53 pubmed publisher
    ..variant in the alanyl-tRNA synthetase (AARS) gene was found to segregate with dominant axonal CMT type 2N (CMT2N) in two French families; however, the functional consequence of this mutation has not been determined...
  45. Yakobov N, Debard S, Fischer F, Senger B, Becker H. Cytosolic aminoacyl-tRNA synthetases: Unanticipated relocations for unexpected functions. Biochim Biophys Acta. 2018;1861:387-400 pubmed publisher
    ..signaling pathways that trigger their relocation and the new functions associated with these relocating cytosolic aaRS. Finally, given that these relocating pools of cytosolic aaRSs participate to a wide range of cellular pathways ..
  46. Shiba K, Ripmaster T, Suzuki N, Nichols R, Plotz P, Noda T, et al. Human alanyl-tRNA synthetase: conservation in evolution of catalytic core and microhelix recognition. Biochemistry. 1995;34:10340-9 pubmed
    ..Thus, the region of close sequence similarity may be a consequence of strong selective pressure to conserve the acceptor helix G3:U70 base pair as an RNA signal for alanine. ..
  47. Sang Lee J, Gyu Park S, Park H, Seol W, Lee S, Kim S. Interaction network of human aminoacyl-tRNA synthetases and subunits of elongation factor 1 complex. Biochem Biophys Res Commun. 2002;291:158-64 pubmed
    ..These data suggest that a systematic interaction network may exist between mammalian ARSs and EF-1 subunits probably to enhance the efficiency of in vivo protein synthesis. ..
  48. Zhao Z, Hashiguchi A, Hu J, Sakiyama Y, Okamoto Y, Tokunaga S, et al. Alanyl-tRNA synthetase mutation in a family with dominant distal hereditary motor neuropathy. Neurology. 2012;78:1644-9 pubmed publisher
    ..2677G>A (p.D893N) of alanyl-tRNA synthetase (AARS), which was not found in the 4 unaffected members and control subjects...
  49. Sun L, Song Y, Blocquel D, Yang X, Schimmel P. Two crystal structures reveal design for repurposing the C-Ala domain of human AlaRS. Proc Natl Acad Sci U S A. 2016;113:14300-14305 pubmed
    ..b>AlaRS is the single exception, with an appended C-terminal domain (C-Ala) that is conserved from prokaryotes to humans ..
  50. Roberts L, Nossek C, Greenberg L, Ramesar R. Stargardt macular dystrophy: common ABCA4 mutations in South Africa--establishment of a rapid genetic test and relating risk to patients. Mol Vis. 2012;18:280-9 pubmed
    ..a mechanism for identifying common disease-causing mutations in subjects with ABCA4-associated retinopathies (AARs)...
  51. Liu Y, Satz J, Vo M, Nangle L, Schimmel P, Ackerman S. Deficiencies in tRNA synthetase editing activity cause cardioproteinopathy. Proc Natl Acad Sci U S A. 2014;111:17570-5 pubmed publisher
    ..Reduced translational fidelity, caused by a hypomorphic mutation in the editing domain of alanyl-tRNA synthetase (AlaRS), resulted in accumulation of misfolded proteins in specific mouse neurons...
  52. Caetano Anolles G, Kim K, Caetano Anolles D. The phylogenomic roots of modern biochemistry: origins of proteins, cofactors and protein biosynthesis. J Mol Evol. 2012;74:1-34 pubmed publisher
    ..Ancient forms of aminoacyl-tRNA synthetase (aaRS) catalytic domains and ancient non-ribosomal protein synthetase (NRPS) modules gave rise to primordial protein ..
  53. Simons C, Griffin L, Helman G, Golas G, Pizzino A, Bloom M, et al. Loss-of-function alanyl-tRNA synthetase mutations cause an autosomal-recessive early-onset epileptic encephalopathy with persistent myelination defect. Am J Hum Genet. 2015;96:675-81 pubmed publisher
    ..associated with an autosomal-dominant inherited form of axonal neuropathy, Charcot-Marie-Tooth disease type 2N (CMT2N)...
  54. Sun L, Gomes A, He W, Zhou H, Wang X, Pan D, et al. Evolutionary Gain of Alanine Mischarging to Noncognate tRNAs with a G4:U69 Base Pair. J Am Chem Soc. 2016;138:12948-12955 pubmed
    ..Yet, deliberate modifications of translational fidelity can be beneficial. Here we found human and not E. coli AlaRS has an intrinsic capacity for mispairing alanine onto nonalanyl-tRNAs including tRNACys...
  55. Carter C, Wills P. Interdependence, Reflexivity, Fidelity, Impedance Matching, and the Evolution of Genetic Coding. Mol Biol Evol. 2017;: pubmed publisher
    ..thought to have required ribozymes whose functions were taken over by polypeptide aminoacyl-tRNA synthetases (aaRS)...
  56. Ripmaster T, Shiba K, Schimmel P. Wide cross-species aminoacyl-tRNA synthetase replacement in vivo: yeast cytoplasmic alanine enzyme replaced by human polymyositis serum antigen. Proc Natl Acad Sci U S A. 1995;92:4932-6 pubmed
    ..U70 system of recognition is sufficient to yield accurate aminoacylation in vivo across wide species distances. ..
  57. Nichols R, Pai S, Ge Q, Targoff I, Plotz P, Liu P. Localization of two human autoantigen genes by PCR screening and in situ hybridization--glycyl-tRNA synthetase locates to 7p15 and alanyl-tRNA synthetase locates to 16q22. Genomics. 1995;30:131-2 pubmed
  58. Nakayama T, Wu J, Galvin Parton P, Weiss J, Andriola M, Hill R, et al. Deficient activity of alanyl-tRNA synthetase underlies an autosomal recessive syndrome of progressive microcephaly, hypomyelination, and epileptic encephalopathy. Hum Mutat. 2017;38:1348-1354 pubmed publisher
    ..Although alanyl-tRNA synthetase (AARS) is a synthetase implicated in a wide range of neurological disorders from Charcot-Marie-Tooth disease to infantile ..
  59. Lovato M, Chihade J, Schimmel P. Translocation within the acceptor helix of a major tRNA identity determinant. EMBO J. 2001;20:4846-53 pubmed
    ..translocated G:U and the adjacent G3:C70 are major determinants for recognition by Dm mt alanyl-tRNA synthetase (AlaRS). Additionally, G:U at the 3:70 position serves as an anti-determinant for Dm mt AlaRS...
  60. Grube C, Roy H. A continuous assay for monitoring the synthetic and proofreading activities of multiple aminoacyl-tRNA synthetases for high-throughput drug discovery. RNA Biol. 2017;: pubmed publisher
    ..The aaRS editing site hydrolyzes misacylated products formed when a non-cognate amino acid is used during tRNA charging...
  61. Masoudi M, Derakhshan N, Ghaffarpasand F, Sadeghpour T. Management of Pediatric Atlantoaxial Rotatory Subluxation with a Simple Handmade Cervical Traction Device: Doing More with Less. World Neurosurg. 2017;106:355-358 pubmed publisher
    To introduce a novel traction device for management of pediatric atlanto-axial rotatory subluxation (AARS) in source limiting areas. Atlanto-axial (C1-C2) joint is accountable for up to two third of total axial craniocervical rotation...
  62. Dohrn M, Glöckle N, Mulahasanovic L, Heller C, Mohr J, Bauer C, et al. Frequent genes in rare diseases: panel-based next generation sequencing to disclose causal mutations in hereditary neuropathies. J Neurochem. 2017;143:507-522 pubmed publisher
    ..pathogenic variants in HINT1, HSPB1, NEFL, PRX, IGHMBP2, NDRG1, TTR, EGR2, FIG4, GDAP1, LMNA, LRSAM1, POLG, TRPV4, AARS, BIC2, DHTKD1, FGD4, HK1, INF2, KIF5A, PDK3, REEP1, SBF1, SBF2, SCN9A, and SPTLC2 with a declining frequency...
  63. Vijayakumar R, Tripathi T. Soluble expression and purification of a full-length asparaginyl tRNA synthetase from Fasciola gigantica. Protein Expr Purif. 2018;143:9-13 pubmed publisher
    ..It causes fascioliasis that infects the liver of various mammals, including humans. Aminoacyl tRNA synthetases (AARS) catalyze the first step of protein synthesis...
  64. He X, Li S, Liu B, Susperreguy S, Formoso K, Yao J, et al. Major contribution of the 3/6/7 class of TRPC channels to myocardial ischemia/reperfusion and cellular hypoxia/reoxygenation injuries. Proc Natl Acad Sci U S A. 2017;114:E4582-E4591 pubmed publisher
    ..subjected to 9-h hypoxia followed by 6-h reoxygenation (H/R), and analyzing changes occurring in areas-at-risk (AARs) of murine hearts subjected to a 30-min ischemia followed by 24-h reperfusion (I/R) protocol, we found: (i) ..
  65. Bánsági B, Antoniadi T, Burton Jones S, Murphy S, McHugh J, Alexander M, et al. Genotype/phenotype correlations in AARS-related neuropathy in a cohort of patients from the United Kingdom and Ireland. J Neurol. 2015;262:1899-908 pubmed publisher
    ..To date six families have been reported worldwide with dominant missense alanyl-tRNA synthetase (AARS) mutations leading to clinically heterogeneous axonal neuropathies...
  66. Wang L. Engineering the Genetic Code in Cells and Animals: Biological Considerations and Impacts. Acc Chem Res. 2017;50:2767-2775 pubmed publisher
    ..Orthogonal components including tRNA and aminoacyl-tRNA synthetase (aaRS) are expressed in live cells to decode a unique codon (often the amber stop codon UAG) as the desired Uaa...
  67. Latour P, Thauvin Robinet C, Baudelet Méry C, Soichot P, Cusin V, Faivre L, et al. A major determinant for binding and aminoacylation of tRNA(Ala) in cytoplasmic Alanyl-tRNA synthetase is mutated in dominant axonal Charcot-Marie-Tooth disease. Am J Hum Genet. 2010;86:77-82 pubmed publisher
    ..Arg329His), affecting a totally conserved amino acid in the helical domain of cytoplasmic alanyl-tRNA synthetase (AlaRS)...
  68. Ferro I, Liebeton K, Ignatova Z. Growth-Rate Dependent Regulation of tRNA Level and Charging in Bacillus licheniformis. J Mol Biol. 2017;429:3102-3112 pubmed publisher
    ..level is kept relatively constant through riboswitch-regulated expression of the cognate aminoacyl-tRNA-synthetase (AARS)...
  69. Motley W, Griffin L, Mademan I, Baets J, De Vriendt E, De Jonghe P, et al. A novel AARS mutation in a family with dominant myeloneuropathy. Neurology. 2015;84:2040-7 pubmed publisher
    ..304G>C (p.Gly102Arg) in the alanyl-tRNA synthetase (AARS) gene; this allele was not identified in unaffected individuals or control samples...
  70. Andrade V, Guerra M, Jardim C, Melo F, Silva W, Ortega J, et al. Nucleoplasmic calcium regulates cell proliferation through legumain. J Hepatol. 2011;55:626-635 pubmed publisher
    ..Increased expression of LGMN may be involved in liver carcinogenesis. ..
  71. Francklyn C, Schimmel P. Aminoacylation of RNA minihelices with alanine. Nature. 1989;337:478-81 pubmed
    ..Alanine incorporation requires a single G.U base pair, and occurs in helices that otherwise differ significantly in sequence. Aminoacylation can be achieved with only seven base pairs in the helix. ..
  72. Rathnayake U, Wood W, Hendrickson T. Indirect tRNA aminoacylation during accurate translation and phenotypic mistranslation. Curr Opin Chem Biol. 2017;41:114-122 pubmed publisher
    The fact that most bacteria do not contain a full set of aminoacyl-tRNA synthetases (aaRS) is often underappreciated...
  73. Restelli L, Marques A, Savoini G, Invernizzi G, Carisetti M, Lecchi C, et al. Saturated or unsaturated fat supplemented maternal diets influence omental adipose tissue proteome of suckling goat-kids. Res Vet Sci. 2017;: pubmed publisher
    ..Stearic acid induces changes in a higher number of proteins when compared to fish oil. Eleven proteins, namely AARS, ECl1, PMSC2, CP, HSPA8, GPD1, RPL7, OGDH, RPL24, FGA and RPL5 were decreased in ST-kids only...
  74. Zheng Y, Addy P, Mukherjee R, Chatterjee A. Defining the current scope and limitations of dual noncanonical amino acid mutagenesis in mammalian cells. Chem Sci. 2017;8:7211-7217 pubmed publisher
    ..It would require the use of two different engineered aminoacyl-tRNA synthetase (aaRS)/tRNA pairs, each suppressing a distinct nonsense codon, and which cross-react neither with each other, nor with ..