Publications

• Enzymic formation of ribonucleic acid-amino acid from synthetic aminoacyladenylate and ribonucleic acid.

  WONG K.K., MEISTER A., MOLDAVE K.

  Biochim Biophys Acta 36:531-533(1959) [PubMed] [EuropePMC]

• A recurrent WARS mutation is a novel cause of autosomal dominant distal hereditary motor neuropathy.

  Tsai P.C., Soong B.W., Mademan I., Huang Y.H., Liu C.R., Hsiao C.T., Wu H.T., Liu T.T., Liu Y.T., Tseng Y.T., Lin K.P., Yang U.C., Chung K.W., Choi B.O., Nicholson G.A., Kennerson M.L., Chan C.C., De Jonghe P., Cheng T.H., Liao Y.C., Zuechner S., Baets J., Lee Y.C.

  Distal hereditary motor neuropathy is a heterogeneous group of inherited neuropathies characterized by distal limb muscle weakness and atrophy. Although at least 15 genes have been implicated in distal hereditary motor neuropathy, the genetic causes remain elusive in many families. To identify an ... >> More

  Distal hereditary motor neuropathy is a heterogeneous group of inherited neuropathies characterized by distal limb muscle weakness and atrophy. Although at least 15 genes have been implicated in distal hereditary motor neuropathy, the genetic causes remain elusive in many families. To identify an additional causal gene for distal hereditary motor neuropathy, we performed exome sequencing for two affected individuals and two unaffected members in a Taiwanese family with an autosomal dominant distal hereditary motor neuropathy in which mutations in common distal hereditary motor neuropathy-implicated genes had been excluded. The exome sequencing revealed a heterozygous mutation, c.770A > G (p.His257Arg), in the cytoplasmic tryptophanyl-tRNA synthetase (TrpRS) gene (WARS) that co-segregates with the neuropathy in the family. Further analyses of WARS in an additional 79 Taiwanese pedigrees with inherited neuropathies and 163 index cases from Australian, European, and Korean distal hereditary motor neuropathy families identified the same mutation in another Taiwanese distal hereditary motor neuropathy pedigree with different ancestries and one additional Belgian distal hereditary motor neuropathy family of Caucasian origin. Cell transfection studies demonstrated a dominant-negative effect of the p.His257Arg mutation on aminoacylation activity of TrpRS, which subsequently compromised protein synthesis and reduced cell viability. His257Arg TrpRS also inhibited neurite outgrowth and led to neurite degeneration in the neuronal cell lines and rat motor neurons. Further in vitro analyses showed that the WARS mutation could potentiate the angiostatic activities of TrpRS by enhancing its interaction with vascular endothelial-cadherin. Taken together, these findings establish WARS as a gene whose mutations may cause distal hereditary motor neuropathy and alter canonical and non-canonical functions of TrpRS. << Less

  Brain 140:1252-1266(2017) [PubMed] [EuropePMC]

• Tryptophanyl transfer ribonucleic acid synthetase from bovine pancreas. II. The chemically different subunits.

  Preddie E.C.

  J Biol Chem 244:3958-3968(1969) [PubMed] [EuropePMC]

• The isolation of a tryptophan-activating enzyme from pancreas.

  DAVIE E.W., KONINGSBERGER V.V., LIPMANN F.

  Arch Biochem Biophys 65:21-38(1956) [PubMed] [EuropePMC]

• Aminoacyl-tRNA synthesis.

  Ibba M., Soll D.

  Aminoacyl-tRNAs are substrates for translation and are pivotal in determining how the genetic code is interpreted as amino acids. The function of aminoacyl-tRNA synthesis is to precisely match amino acids with tRNAs containing the corresponding anticodon. This is primarily achieved by the direct a ... >> More

  Aminoacyl-tRNAs are substrates for translation and are pivotal in determining how the genetic code is interpreted as amino acids. The function of aminoacyl-tRNA synthesis is to precisely match amino acids with tRNAs containing the corresponding anticodon. This is primarily achieved by the direct attachment of an amino acid to the corresponding tRNA by an aminoacyl-tRNA synthetase, although intrinsic proofreading and extrinsic editing are also essential in several cases. Recent studies of aminoacyl-tRNA synthesis, mainly prompted by the advent of whole genome sequencing and the availability of a vast body of structural data, have led to an expanded and more detailed picture of how aminoacyl-tRNAs are synthesized. This article reviews current knowledge of the biochemical, structural, and evolutionary facets of aminoacyl-tRNA synthesis. << Less

  Annu Rev Biochem 69:617-650(2000) [PubMed] [EuropePMC]

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