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Page 1
Defects in t(6)A tRNA modification due to GON7 and YRDC mutations lead to Galloway-Mowat syndrome.
Arrondel C, Missoury S, Snoek R, Patat J, Menara G, Collinet B, Liger D, Durand D, Gribouval O, Boyer O, Buscara L, Martin G, Machuca E, Nevo F, Lescop E, Braun DA, Boschat AC, Sanquer S, Guerrera IC, Revy P, Parisot M, Masson C, Boddaert N, Charbit M, Decramer S, Novo R, Macher MA, Ranchin B, Bacchetta J, Laurent A, Collardeau-Frachon S, van Eerde AM, Hildebrandt F, Magen D, Antignac C, van Tilbeurgh H, Mollet G. Arrondel C, et al. Nat Commun. 2019 Sep 3;10(1):3967. doi: 10.1038/s41467-019-11951-x. Nat Commun. 2019. PMID: 31481669 Free PMC article.
Here we show that mutations in YRDC cause an extremely severe form of GAMOS whereas mutations in GON7, encoding the fifth KEOPS subunit, lead to a milder form of the disease. The crystal structure of the GON7/LAGE3/OSGEP subcomplex shows that the intrinsically disor …
Here we show that mutations in YRDC cause an extremely severe form of GAMOS whereas mutations in GON7, encoding the fifth KEOPS subun …
The structural and functional workings of KEOPS.
Beenstock J, Sicheri F. Beenstock J, et al. Nucleic Acids Res. 2021 Nov 8;49(19):10818-10834. doi: 10.1093/nar/gkab865. Nucleic Acids Res. 2021. PMID: 34614169 Free PMC article. Review.
The Kae1 subunit of KEOPS catalyzes the universal and essential tRNA modification N6-threonylcarbamoyl adenosine (t6A), while the auxiliary subunits Cgi121, the kinase/ATPase Bud32, Pcc1 and Gon7 play a supporting role. Kae1 orthologs are also present in bacteria and mitoc …
The Kae1 subunit of KEOPS catalyzes the universal and essential tRNA modification N6-threonylcarbamoyl adenosine (t6A), while the auxiliary …
Crystal structures of the Gon7/Pcc1 and Bud32/Cgi121 complexes provide a model for the complete yeast KEOPS complex.
Zhang W, Collinet B, Graille M, Daugeron MC, Lazar N, Libri D, Durand D, van Tilbeurgh H. Zhang W, et al. Nucleic Acids Res. 2015 Mar 31;43(6):3358-72. doi: 10.1093/nar/gkv155. Epub 2015 Mar 3. Nucleic Acids Res. 2015. PMID: 25735745 Free PMC article.
The yeast KEOPS protein complex comprising Kae1, Bud32, Cgi121, Pcc1 and Gon7 is responsible for the essential tRNA threonylcarbamoyladenosine (t(6)A) modification. ...Gon7 interacts with the same Pcc1 region engaged in the archaeal Pcc1 homodimer. We further show t …
The yeast KEOPS protein complex comprising Kae1, Bud32, Cgi121, Pcc1 and Gon7 is responsible for the essential tRNA threonylcarbamoyl …
The archaeal KEOPS complex possesses a functional Gon7 homolog and has an essential function independent of the cellular t(6)A modification level.
Wu P, Gan Q, Zhang X, Yang Y, Xiao Y, She Q, Ni J, Huang Q, Shen Y. Wu P, et al. mLife. 2023 Jan 8;2(1):11-27. doi: 10.1002/mlf2.12051. eCollection 2023 Mar. mLife. 2023. PMID: 38818338 Free PMC article.
It is composed of Pcc1, Kae1, Bud32, Cgi121, and Gon7 in eukaryotes and is primarily involved in N(6)-threonylcarbamoyl adenosine (t(6)A) modification of transfer RNAs (tRNAs). ...We show that aKEOPS also possesses five subunits, Pcc1, Kae1, Bud32, Cgi121, and Pcc1-like (o …
It is composed of Pcc1, Kae1, Bud32, Cgi121, and Gon7 in eukaryotes and is primarily involved in N(6)-threonylcarbamoyl adenosine (t( …
Proteomic analysis of the human KEOPS complex identifies C14ORF142 as a core subunit homologous to yeast Gon7.
Wan LC, Maisonneuve P, Szilard RK, Lambert JP, Ng TF, Manczyk N, Huang H, Laister R, Caudy AA, Gingras AC, Durocher D, Sicheri F. Wan LC, et al. Nucleic Acids Res. 2017 Jan 25;45(2):805-817. doi: 10.1093/nar/gkw1181. Epub 2016 Nov 29. Nucleic Acids Res. 2017. PMID: 27903914 Free PMC article.
To identify a Gon7 ortholog in multicellular eukaryotes as well as to uncover KEOPS-interacting proteins that may link t(6)A biosynthesis to the diverse set of KEOPS mutant phenotypes, we conducted a proteomic analysis of human KEOPS. ...Further characterization of C14ORF1 …
To identify a Gon7 ortholog in multicellular eukaryotes as well as to uncover KEOPS-interacting proteins that may link t(6)A biosynth …
A paralog of Pcc1 is the fifth core subunit of the KEOPS tRNA-modifying complex in Archaea.
Daugeron MC, Missoury S, Da Cunha V, Lazar N, Collinet B, van Tilbeurgh H, Basta T. Daugeron MC, et al. Nat Commun. 2023 Feb 1;14(1):526. doi: 10.1038/s41467-023-36210-y. Nat Commun. 2023. PMID: 36720870 Free PMC article.
In Archaea and Eukaryotes, the synthesis of a universal tRNA modification, N(6)-threonyl-carbamoyl adenosine (t(6)A), is catalyzed by the KEOPS complex composed of Kae1, Bud32, Cgi121, and Pcc1. A fifth subunit, Gon7, is found only in Fungi and Metazoa. Here, we identify a …
In Archaea and Eukaryotes, the synthesis of a universal tRNA modification, N(6)-threonyl-carbamoyl adenosine (t(6)A), is catalyzed by the KE …
Crystal structure of the human PRPK-TPRKB complex.
Li J, Ma X, Banerjee S, Chen H, Ma W, Bode AM, Dong Z. Li J, et al. Commun Biol. 2021 Feb 5;4(1):167. doi: 10.1038/s42003-021-01683-4. Commun Biol. 2021. PMID: 33547416 Free PMC article.
We constructed a structural model of the human EKC/KEOPS complex, composed of PRPK, TPRKB, OSGEP, LAGE3, and GON7. Disease mutations in PRPK and TPRKB are mapped into the structure, and we show that one mutation, PRPK K238Nfs*2, lost the binding to OSGEP. ...
We constructed a structural model of the human EKC/KEOPS complex, composed of PRPK, TPRKB, OSGEP, LAGE3, and GON7. Disease mutations …
Kae1 of Saccharomyces cerevisiae KEOPS complex possesses ADP/GDP nucleotidase activity.
Li QX, Liu JC, He MH, Zhou JQ. Li QX, et al. Biochem J. 2022 Dec 9;479(23):2433-2447. doi: 10.1042/BCJ20220290. Biochem J. 2022. PMID: 36416748
In budding yeast Saccharomyces cerevisiae, the KEOPS complex (ScKEOPS) consists of five subunits, which are Kae1, Bud32, Cgi121, Pcc1, and Gon7. The KEOPS complex is an ATPase and is required for tRNA N6-threonylcarbamoyladenosine modification, telomere length maintenance, …
In budding yeast Saccharomyces cerevisiae, the KEOPS complex (ScKEOPS) consists of five subunits, which are Kae1, Bud32, Cgi121, Pcc1, and …
Diagnosis delay a family of Galloway-Mowat Syndrome caused by a classical splicing mutation of Lage3.
Chen Y, Yang Y, Yang Y, Rao J, Bai H. Chen Y, et al. BMC Nephrol. 2023 Feb 8;24(1):29. doi: 10.1186/s12882-022-03000-5. BMC Nephrol. 2023. PMID: 36755238 Free PMC article.
BACKGROUND: Galloway-Mowat syndrome (GAMOS) is a group of rare hereditary diseases by the combination of early onset steroid-resistant nephrotic syndrome (SRNS) and microcephaly with brain anomalies caused by WDR73, LAGE3, OSGEP, TP53RK, TPRKB, GON7, WDR4 or NUP133 mutatio …
BACKGROUND: Galloway-Mowat syndrome (GAMOS) is a group of rare hereditary diseases by the combination of early onset steroid-resistant nephr …
KEOPS complex expression in the frontal cortex in major depression and schizophrenia.
Abel ME, Zhang X, Asah SM, Wolfinger A, McCullumsmith RE, O'Donovan SM. Abel ME, et al. World J Biol Psychiatry. 2021 Jul;22(6):446-455. doi: 10.1080/15622975.2020.1821917. Epub 2020 Sep 29. World J Biol Psychiatry. 2021. PMID: 32914678 Free PMC article.
METHODS: We assessed mRNA expression of KEOPS complex subunits TP53RK, TPRKB, GON7, LAGE3, OSGEP, and OSGEP mitochondrial ortholog OSGEPL1 in the dorsolateral prefrontal cortex (DLPFC) of subjects with MDD, schizophrenia and matched non-psychiatrically ill controls (n = 20 …
METHODS: We assessed mRNA expression of KEOPS complex subunits TP53RK, TPRKB, GON7, LAGE3, OSGEP, and OSGEP mitochondrial ortholog OS …
18 results