Glutamyl-tRNA(Gln) amidotransferase

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spinqualitylabelsall models Caption for this structure Show:Asymmetric Unit Biological Assembly Drag the structure with the mouse to rotate   Export Animated Image Contents 1 Function 2 Disease 3 Relevance 4 Structural highlights 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]. Relevance Structural highlights

3D structures of glutamyl-tRNA(Gln) amidotransferase

Updated on 03-May-2020

References

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