Glutamyl-tRNA(Gln) amidotransferase

Function

Glutamyl-tRNA(Gln) amidotransferase (Glu-ADT) and Aspartyl/glutamyl-tRNA(Asn/Gln) amidotransferase (Asp/Glu-ADT) are the 2nd enzyme in the pathway which convert Glu-tRNA or Asp-tRNA to Gln-tRNA or Asn-tRNA in organisms which lack Gln-tRNA synthetase or Asn-tRNA ^[1].

Disease

Mutations in Glu-ADT in families with genetic defect show a lethal metabolic cardiomyopathy syndrome^[2].

Structural highlights

The 3D structure of yeast Glu-ADT shows the . tRNA binds to the by homology modeling^[3].

Yeast Glu-ADT subunit A (cyan) + subunit B (green) + subunit F (pink) complex with Gln and NO3- ion (PDB code 4n0i)

Drag the structure with the mouse to rotate

3D structures of glutamyl-tRNA(Gln) amidotransferase3D structures of glutamyl-tRNA(Gln) amidotransferase

Updated on 04-August-2020

ReferencesReferences

↑ Curnow AW, Tumbula DL, Pelaschier JT, Min B, Soll D. Glutamyl-tRNA(Gln) amidotransferase in Deinococcus radiodurans may be confined to asparagine biosynthesis. Proc Natl Acad Sci U S A. 1998 Oct 27;95(22):12838-43. doi:, 10.1073/pnas.95.22.12838. PMID:9789001 doi:http://dx.doi.org/10.1073/pnas.95.22.12838
↑ Friederich MW, Timal S, Powell CA, Dallabona C, Kurolap A, Palacios-Zambrano S, Bratkovic D, Derks TGJ, Bick D, Bouman K, Chatfield KC, Damouny-Naoum N, Dishop MK, Falik-Zaccai TC, Fares F, Fedida A, Ferrero I, Gallagher RC, Garesse R, Gilberti M, Gonzalez C, Gowan K, Habib C, Halligan RK, Kalfon L, Knight K, Lefeber D, Mamblona L, Mandel H, Mory A, Ottoson J, Paperna T, Pruijn GJM, Rebelo-Guiomar PF, Saada A, Sainz B Jr, Salvemini H, Schoots MH, Smeitink JA, Szukszto MJ, Ter Horst HJ, van den Brandt F, van Spronsen FJ, Veltman JA, Wartchow E, Wintjes LT, Zohar Y, Fernandez-Moreno MA, Baris HN, Donnini C, Minczuk M, Rodenburg RJ, Van Hove JLK. Pathogenic variants in glutamyl-tRNA(Gln) amidotransferase subunits cause a lethal mitochondrial cardiomyopathy disorder. Nat Commun. 2018 Oct 3;9(1):4065. doi: 10.1038/s41467-018-06250-w. PMID:30283131 doi:http://dx.doi.org/10.1038/s41467-018-06250-w
↑ Araiso Y, Huot JL, Sekiguchi T, Frechin M, Fischer F, Enkler L, Senger B, Ishitani R, Becker HD, Nureki O. Crystal structure of Saccharomyces cerevisiae mitochondrial GatFAB reveals a novel subunit assembly in tRNA-dependent amidotransferases. Nucleic Acids Res. 2014 Apr 1. PMID:24692665 doi:http://dx.doi.org/10.1093/nar/gku234

Proteopedia Page Contributors and Editors (what is this?)Proteopedia Page Contributors and Editors (what is this?)

Michal Harel, Alexander Berchansky

[1] Curnow AW, Tumbula DL, Pelaschier JT, Min B, Soll D. Glutamyl-tRNA(Gln) amidotransferase in Deinococcus radiodurans may be confined to asparagine biosynthesis. Proc Natl Acad Sci U S A. 1998 Oct 27;95(22):12838-43. doi:, 10.1073/pnas.95.22.12838. PMID:9789001 doi:http://dx.doi.org/10.1073/pnas.95.22.12838

[2] Friederich MW, Timal S, Powell CA, Dallabona C, Kurolap A, Palacios-Zambrano S, Bratkovic D, Derks TGJ, Bick D, Bouman K, Chatfield KC, Damouny-Naoum N, Dishop MK, Falik-Zaccai TC, Fares F, Fedida A, Ferrero I, Gallagher RC, Garesse R, Gilberti M, Gonzalez C, Gowan K, Habib C, Halligan RK, Kalfon L, Knight K, Lefeber D, Mamblona L, Mandel H, Mory A, Ottoson J, Paperna T, Pruijn GJM, Rebelo-Guiomar PF, Saada A, Sainz B Jr, Salvemini H, Schoots MH, Smeitink JA, Szukszto MJ, Ter Horst HJ, van den Brandt F, van Spronsen FJ, Veltman JA, Wartchow E, Wintjes LT, Zohar Y, Fernandez-Moreno MA, Baris HN, Donnini C, Minczuk M, Rodenburg RJ, Van Hove JLK. Pathogenic variants in glutamyl-tRNA(Gln) amidotransferase subunits cause a lethal mitochondrial cardiomyopathy disorder. Nat Commun. 2018 Oct 3;9(1):4065. doi: 10.1038/s41467-018-06250-w. PMID:30283131 doi:http://dx.doi.org/10.1038/s41467-018-06250-w

[3] Araiso Y, Huot JL, Sekiguchi T, Frechin M, Fischer F, Enkler L, Senger B, Ishitani R, Becker HD, Nureki O. Crystal structure of Saccharomyces cerevisiae mitochondrial GatFAB reveals a novel subunit assembly in tRNA-dependent amidotransferases. Nucleic Acids Res. 2014 Apr 1. PMID:24692665 doi:http://dx.doi.org/10.1093/nar/gku234

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