Glutamyl-tRNA(Gln) amidotransferase

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Mutations in Glu-ADT in families with genetic defect show a lethal metabolic cardiomyopathy syndrome<ref>PMID:30283131</ref>.
Mutations in Glu-ADT in families with genetic defect show a lethal metabolic cardiomyopathy syndrome<ref>PMID:30283131</ref>.
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== Relevance ==
 
== Structural highlights ==
== Structural highlights ==
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The 3D structure of yeast Glu-ADT shows the binding of Gln to subunit A. tRNA binds to the C-terminal of subunit B by homology modeling<ref>PMID:24692665</ref>.
</StructureSection>
</StructureSection>

Revision as of 10:09, 3 May 2020

Yeast Glu-ADT subunit A (grey) + 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) 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
  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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Michal Harel, Alexander Berchansky

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