Article

Structure of the human KEOPS/tRNA complex and characterization of pathogenic variants responsible for the Galloway Mowat syndrome

2026-03-03

Abstract excerpt

N 6 -threonyl-carbamoylation of adenosine 37 of ANN-type tRNAs (t 6 A) is a universal modification essential for translational accuracy and efficiency. The t 6 A pathway uses two sequentially acting enzymes, YRDC and OSGEP, the latter being a subunit of the multiprotein KEOPS complex. Structures of the subunits and subcomplexes of human KEOPS are known, but knowledge on the detailed interactions with tRNA is la...

Topics

Open a Topic to create a Post that cites this publication.

Identifiers and source

Literature Corpus work: b95f61d4-ab98-5ad5-958e-5820162556ba
DOI: 10.64898/2026.03.03.709218

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

Structure of the human KEOPS/tRNA complex and characterization of pathogenic variants responsible for the Galloway Mowat syndromeDOI 10.64898/2026.03.03.709218Read

tRNA N6-adenosine threonylcarbamoyltransferase defect due to KAE1/TCS3 (OSGEP) mutation manifest by neurodegeneration and renal tubulopathy.Semantic proximity Read

The structural and functional workings of KEOPSSemantic proximity Read

A paralog of Pcc1 is the fifth core subunit of KEOPS complex in ArchaeaSemantic proximity Read

Proteomic analysis of the human KEOPS complex identifies C14ORF142 as a core subunit homologous to yeast Gon7Semantic proximity Read

Catalytic and regulatory basis of tRNA t6A modification by the KEOPS complex.Semantic proximity Read

Gcn4 misregulation reveals a direct role for the evolutionary conserved EKC/KEOPS in the t6A modification of tRNAs.Semantic proximity Read

Commonality and diversity in tRNA substrate recognition in t6A biogenesis by eukaryotic KEOPSsSemantic proximity Read

Mutations in KEOPS-complex genes cause nephrotic syndrome with primary microcephaly.Semantic proximity Read

Loss of Elongator- and KEOPS-Dependent tRNA Modifications Leads to Severe Growth Phenotypes and Protein Aggregation in Yeast.Semantic proximity Read

The Biology of tRNA t6A Modification and Hypermodifications—Biogenesis and Disease RelevanceSemantic proximity Read

Loss of Elongator- and KEOPS-Dependent tRNA Modifications Leads to Severe Growth Phenotypes and Protein Aggregation in YeastSemantic proximity Read

Jekyll & Hyde: evolution of a superfamily.Semantic proximity Read