Gene Symbol: Ate1
Description: arginyltransferase 1
Alias: AI225793, AW547406, arginyl-tRNA--protein transferase 1, R-transferase 1, arginine-tRNA--protein transferase 1
Species: mouse
Products:     Ate1

Top Publications

  1. Kwon Y, Kashina A, Varshavsky A. Alternative splicing results in differential expression, activity, and localization of the two forms of arginyl-tRNA-protein transferase, a component of the N-end rule pathway. Mol Cell Biol. 1999;19:182-93 pubmed
    ..We isolated cDNA encoding the 516-residue mouse R-transferase, ATE1p, and found two species, termed Ate1-1 and Ate1-2...
  2. Hu R, Brower C, Wang H, Davydov I, Sheng J, Zhou J, et al. Arginyltransferase, its specificity, putative substrates, bidirectional promoter, and splicing-derived isoforms. J Biol Chem. 2006;281:32559-73 pubmed
    ..The conjugation of Arg is mediated by arginyltransferase, encoded by ATE1. Through its regulated degradation of specific proteins, the arginylation branch of the N-end rule pathway mediates,..
  3. Wong C, Xu T, Rai R, Bailey A, Yates J, Wolf Y, et al. Global analysis of posttranslational protein arginylation. PLoS Biol. 2007;5:e258 pubmed
    ..The results of our study suggest that protein arginylation is a general mechanism for regulation of protein structure and function and outline the potential role of protein arginylation in cell metabolism and embryonic development. ..
  4. Kwon Y, Kashina A, Davydov I, Hu R, An J, Seo J, et al. An essential role of N-terminal arginylation in cardiovascular development. Science. 2002;297:96-9 pubmed
    ..The mouse ATE1 gene encodes a family of Arg-tRNA-protein transferases (R-transferases) that mediate N-terminal arginylation...
  5. Rai R, Kashina A. Identification of mammalian arginyltransferases that modify a specific subset of protein substrates. Proc Natl Acad Sci U S A. 2005;102:10123-8 pubmed
    Posttranslational N-terminal protein arginylation, mediated by Arg-tRNA-protein transferase 1 (ATE1), is essential for cardiovascular development and angiogenesis in mammals but is nonessential in yeast...
  6. Hu R, Sheng J, Qi X, Xu Z, Takahashi T, Varshavsky A. The N-end rule pathway as a nitric oxide sensor controlling the levels of multiple regulators. Nature. 2005;437:981-6 pubmed
    ..Three amino (N)-terminal residues--aspartate, glutamate and cysteine--are arginylated by ATE1-encoded arginyl-transferases...
  7. Wang J, Han X, Saha S, Xu T, Rai R, Zhang F, et al. Arginyltransferase is an ATP-independent self-regulating enzyme that forms distinct functional complexes in vivo. Chem Biol. 2011;18:121-30 pubmed publisher
    Posttranslational arginylation mediated by arginyl transferase (ATE1) plays an important role in cardiovascular development, cell motility, and regulation of cytoskeleton and metabolic enzymes...
  8. Rai R, Mushegian A, Makarova K, Kashina A. Molecular dissection of arginyltransferases guided by similarity to bacterial peptidoglycan synthases. EMBO Rep. 2006;7:800-5 pubmed
    ..cardiovascular development and angiogenesis in mice and is mediated by arginyl-transfer RNA-protein transferases Ate1-a functionally conserved but poorly understood class of enzymes...
  9. Rai R, Wong C, Xu T, Leu N, Dong D, Guo C, et al. Arginyltransferase regulates alpha cardiac actin function, myofibril formation and contractility during heart development. Development. 2008;135:3881-9 pubmed publisher
    Post-translational arginylation mediated by arginyltransferase (Ate1) is essential for cardiovascular development and angiogenesis in mammals and directly affects myocardium structure in the developing heart...

More Information


  1. Leu N, Kurosaka S, Kashina A. Conditional Tek promoter-driven deletion of arginyltransferase in the germ line causes defects in gametogenesis and early embryonic lethality in mice. PLoS ONE. 2009;4:e7734 pubmed publisher
    Posttranslational protein arginylation mediated by Ate1 is essential for cardiovascular development, actin cytoskeleton functioning, and cell migration...
  2. Piatkov K, Brower C, Varshavsky A. The N-end rule pathway counteracts cell death by destroying proapoptotic protein fragments. Proc Natl Acad Sci U S A. 2012;109:E1839-47 pubmed publisher
    ..In conjunction with other apoptosis-suppressing circuits, the Arg/N-end rule pathway contributes to thresholds that prevent a transient or otherwise weak proapoptotic signal from reaching the point of commitment to apoptosis. ..
  3. Kurosaka S, Leu N, Zhang F, Bunte R, Saha S, Wang J, et al. Arginylation-dependent neural crest cell migration is essential for mouse development. PLoS Genet. 2010;6:e1000878 pubmed publisher
    ..Knockout of arginyltransferase (Ate1) in mice leads to embryonic lethality and severe heart defects that are reminiscent of cell migration-dependent ..
  4. Rai R, Zhang F, Colavita K, Leu N, Kurosaka S, Kumar A, et al. Arginyltransferase suppresses cell tumorigenic potential and inversely correlates with metastases in human cancers. Oncogene. 2016;35:4058-68 pubmed publisher
    Arginylation is an emerging post-translational modification mediated by arginyltransferase (ATE1) that is essential for mammalian embryogenesis and regulation of the cytoskeleton...
  5. Lian L, Suzuki A, Hayes V, Saha S, Han X, Xu T, et al. Loss of ATE1-mediated arginylation leads to impaired platelet myosin phosphorylation, clot retraction, and in vivo thrombosis formation. Haematologica. 2014;99:554-60 pubmed publisher
    Protein arginylation by arginyl-transfer RNA protein transferase (ATE1) is emerging as a regulator protein function that is reminiscent of phosphorylation...
  6. Saha S, Kashina A. Posttranslational arginylation as a global biological regulator. Dev Biol. 2011;358:1-8 pubmed publisher protein arginylation - posttranslational tRNA-mediated addition of arginine, to proteins by arginyltransferase, ATE1. Recent studies show that arginylation is essential for embryogenesis in many organisms and that it regulates such ..
  7. Leite F, Minozzo F, Kalganov A, Cornachione A, Cheng Y, Leu N, et al. Reduced passive force in skeletal muscles lacking protein arginylation. Am J Physiol Cell Physiol. 2016;310:C127-35 pubmed publisher
    ..were decreased in myofibrils and single sarcomeres isolated from muscles lacking arginyl-tRNA-protein transferase (ATE1). 2) Mass spectrometry revealed five sites for arginylation within titin molecules...
  8. Liu Y, Liu C, Chang Z, Wadas B, Brower C, Song Z, et al. Degradation of the Separase-cleaved Rec8, a Meiotic Cohesin Subunit, by the N-end Rule Pathway. J Biol Chem. 2016;291:7426-38 pubmed publisher
    The Ate1 arginyltransferase (R-transferase) is a component of the N-end rule pathway, which recognizes proteins containing N-terminal degradation signals called N-degrons, polyubiquitylates these proteins, and thereby causes their ..
  9. Wadas B, Piatkov K, Brower C, Varshavsky A. Analyzing N-terminal Arginylation through the Use of Peptide Arrays and Degradation Assays. J Biol Chem. 2016;291:20976-20992 pubmed
    N?-terminal arginylation (Nt-arginylation) of proteins is mediated by the Ate1 arginyltransferase (R-transferase), a component of the Arg/N-end rule pathway...
  10. Wang J, Pavlyk I, Vedula P, Sterling S, Leu N, Dong D, et al. Arginyltransferase ATE1 is targeted to the neuronal growth cones and regulates neurite outgrowth during brain development. Dev Biol. 2017;430:41-51 pubmed publisher
    Arginylation is an emerging protein modification mediated by arginyltransferase ATE1, shown to regulate embryogenesis and actin cytoskeleton, however its functions in different physiological systems are not well understood...
  11. Sjögren B, Swaney S, Neubig R. FBXO44-Mediated Degradation of RGS2 Protein Uniquely Depends on a Cullin 4B/DDB1 Complex. PLoS ONE. 2015;10:e0123581 pubmed publisher
    ..Pharmacological targeting of this mechanism provides a novel therapeutic approach to hypertension, anxiety, and other diseases associated with RGS2 dysregulation. ..
  12. Kurosaka S, Leu N, Pavlov I, Han X, Ribeiro P, Xu T, et al. Arginylation regulates myofibrils to maintain heart function and prevent dilated cardiomyopathy. J Mol Cell Cardiol. 2012;53:333-41 pubmed publisher
    Protein arginylation mediated by arginyltransferase (ATE1) is essential for heart formation during embryogenesis, however its cell-autonomous role in cardiomyocytes and the differentiated heart muscle has never been investigated...
  13. Kim E, Kim S, Lee J, Kwon Y, Lee M. Ablation of Arg-tRNA-protein transferases results in defective neural tube development. BMB Rep. 2016;49:443-8 pubmed
    ..the N-end rule pathway is a ubiquitin-mediated proteolytic system in which post-translational conjugation of Arg by ATE1-encoded Arg-tRNA-protein transferase to N-terminal Asp, Glu, or oxidized Cys residues generates essential ..
  14. Carpio M, López Sambrooks C, Durand E, Hallak M. The arginylation-dependent association of calreticulin with stress granules is regulated by calcium. Biochem J. 2010;429:63-72 pubmed publisher
    ..In the present study, we show that cytoplasmic CRT (calreticulin) is arginylated by ATE1 (arginyl-tRNA protein transferase)...
  15. Lee M, Kim D, Zakrzewska A, Yoo Y, Kim S, Kim S, et al. Characterization of arginylation branch of N-end rule pathway in G-protein-mediated proliferation and signaling of cardiomyocytes. J Biol Chem. 2012;287:24043-52 pubmed publisher
    ..In eukaryotes, the major way to generate N-degrons is through arginylation by ATE1 arginyl-tRNA-protein transferases, which transfer Arg from aminoacyl-tRNA to N-terminal Asp and Glu (and Cys as ..
  16. Brower C, Rosen C, Jones R, Wadas B, Piatkov K, Varshavsky A. Liat1, an arginyltransferase-binding protein whose evolution among primates involved changes in the numbers of its 10-residue repeats. Proc Natl Acad Sci U S A. 2014;111:E4936-45 pubmed publisher
    The arginyltransferase Ate1 is a component of the N-end rule pathway, which recognizes proteins containing N-terminal degradation signals called N-degrons, polyubiquitylates these proteins, and thereby causes their degradation by the ..
  17. Ribeiro P, Ribeiro J, Minozzo F, Pavlov I, Leu N, Kurosaka S, et al. Contractility of myofibrils from the heart and diaphragm muscles measured with atomic force cantilevers: effects of heart-specific deletion of arginyl-tRNA-protein transferase. Int J Cardiol. 2013;168:3564-71 pubmed publisher
    ..These results suggest a potential compensatory mechanism by which the diaphragm works under loading conditions in the ?-MHCAte1 chronic heart failure model. ..
  18. Piatkov K, Colnaghi L, Bekes M, Varshavsky A, Huang T. The auto-generated fragment of the Usp1 deubiquitylase is a physiological substrate of the N-end rule pathway. Mol Cell. 2012;48:926-33 pubmed publisher
    ..Thus, in addition to its other functions in DNA repair and chromosome segregation, the Arg/N-end rule pathway regulates genomic stability through the degradation-mediated control of the autocleaved Usp1 deubiquitylase. ..
  19. Saha S, Wang J, Buckley B, Wang Q, Lilly B, Chernov M, et al. Small molecule inhibitors of arginyltransferase regulate arginylation-dependent protein degradation, cell motility, and angiogenesis. Biochem Pharmacol. 2012;83:866-73 pubmed publisher
    Posttranslational arginylation mediated by arginyltransferase (ATE1) is an emerging major regulator of embryogenesis and cell physiology...
  20. Kwon Y, Balogh S, Davydov I, Kashina A, Yoon J, Xie Y, et al. Altered activity, social behavior, and spatial memory in mice lacking the NTAN1p amidase and the asparagine branch of the N-end rule pathway. Mol Cell Biol. 2000;20:4135-48 pubmed
  21. Zhang F, Patel D, Colavita K, Rodionova I, Buckley B, Scott D, et al. Arginylation regulates purine nucleotide biosynthesis by enhancing the activity of phosphoribosyl pyrophosphate synthase. Nat Commun. 2015;6:7517 pubmed publisher
    ..This mechanism promotes arginylation-specific degradation of PRPS1 and selective retention of arginylated PRPS2 in vivo. We therefore demonstrate that arginylation affects both the activity and stability of a major metabolic enzyme. ..
  22. Wang J, Han X, Wong C, Cheng H, Aslanian A, Xu T, et al. Arginyltransferase ATE1 catalyzes midchain arginylation of proteins at side chain carboxylates in vivo. Chem Biol. 2014;21:331-7 pubmed publisher
    Arginylation is an emerging posttranslational modification mediated by Arg-tRNA-protein-transferase (ATE1)...
  23. Brower C, Varshavsky A. Ablation of arginylation in the mouse N-end rule pathway: loss of fat, higher metabolic rate, damaged spermatogenesis, and neurological perturbations. PLoS ONE. 2009;4:e7757 pubmed publisher
    ..N-terminal arginylation is mediated by the Ate1 arginyltransferase, whose physiological substrates include the Rgs4, Rgs5 and Rgs16 regulators of G proteins...
  24. Kumar A, Birnbaum M, Patel D, Morgan W, Singh J, Barrientos A, et al. Posttranslational arginylation enzyme Ate1 affects DNA mutagenesis by regulating stress response. Cell Death Dis. 2016;7:e2378 pubmed publisher
    Arginyltransferase 1 (Ate1) mediates protein arginylation, a poorly understood protein posttranslational modification (PTM) in eukaryotic cells...