tRNA aminoacylation (Homo sapiens)

From WikiPathways

Revision as of 12:59, 21 August 2015 by ReactomeTeam (Talk | contribs)
Jump to: navigation, search
7, 11, 13, 20, 26...332617353321810838365, 9, 454035211631384215324, 14, 43123034272437181723, 4122, 25291, 24mitochondrial matrixcytosolH2OYARS dimertRNA(Tyr)L-AsnQARS Ala-tRNA(Ala)ATPAMPtRNA(Phe)AMPATPATPMARS KARS FARSB MARS ATPPPiATPAARSFARS A2B2 tetramerKARS DARS2 dimerL-PheTARS2(1-?)IARS2KARS AIMP1(1-312) PPiAIMP2 Met-tRNA(Met)EPRS PPiHARS2IARS AMPArg-tRNA(Arg)PPitRNA(His)Tyr-tRNA(Tyr)Asp-tRNA(Asp)AMPATPSerWARS PPiWARS2IARS AMPL-ProVARS2LARS Thr-tRNA(Thr)L-MetAMPCys-tRNA(Cys)DARS ATPQARS L-AlaQARS L-HisL-ArgLARS PPiEPRS ATPAMPtRNA(Phe)ATPIARS PPiGARS dimerYARS aminoacyl-tRNAsynthetasemultienzyme complextRNA(Asp)CARS2(1-?)Phe-tRNA(Phe)L-TyrAMPDARS MARS SARS ATPRARS ATPATPAMPtRNA(Thr)Trp-tRNA(Trp)GluPPiAIMP2 L-TrpDARS PPiLARS CARS Leu-tRNA(Leu)Gly-tRNA(Gly)tRNA(Gly)PPiATPATPIARS ATPAsn-tRNA(Asn)EEF1E1 EEF1E1 tRNA(Val)L-ValL-ThrL-TyrMARS ATPAIMP1(1-312) tRNA(Ser)Val-tRNA(Val)Arg-tRNA(Arg)PiQARS Mg2+L-ArgH2OPPiL-HisPPiDARS2 PARS2PPA2 dimerLARS AIMP2 NARStRNA(Ile)L-GlnDARS L-CystRNA(Asn)L-LeutRNA(Cys)RARS RARS L-AsnATPHis-tRNA(His)ATPVal-tRNA(Val)PPA2 GARS dimerPPiPPiPPiEPRS AMPtRNA(Gly)tRNA(Glu)aminoacyl-tRNAsynthetasemultienzyme complexNARS2ATPL-PheATPKARS IARS PPiATPHARSAIMP1(1-312) PPiAMPPPiLARS Met-tRNA(Met)WARS dimertRNA(Lys)LARS RARS Cys-tRNA(Cys)tRNA(Met)ATPGARS(55-739) PPitRNA(Ser)ATPAIMP1(1-312) His-tRNA(His)PPitRNA(Leu)tRNA(Ala)AMPRARS2AMPtRNA(Trp)AMPAIMP2 IARS KARS AIMP1(1-312) SARS dimerATPIARS EPRS EPRS tRNA(Asn)aminoacyl-tRNAsynthetasemultienzyme complexAMPEEF1E1 Gln-tRNA(Gln)DARS Leu-tRNA(Leu)AMPKARS AMPL-SerKARS KARS DARS PPiPhe-tRNA(Phe)ATPPro-tRNA(Pro)AMPL-GlnLys-tRNA(Lys)DARS tRNA(Lys)QARS AMPtRNA(His)PPiPPiAMPPPiCARS dimerPPitRNA(Trp)KARS dimerMg2+RARS Ile-tRNA(Ile)tRNA(Glu)QARSAIMP1(1-312) Pro-tRNA(Pro)aminoacyl-tRNAsynthetasemultienzyme complextRNA(Met)L-TrpATPL-AspATPQARS MARS2ATPIARS tRNA(Arg)ATPAMPGlyAMPL-AlaEPRS VARSGln-tRNA(Gln)Glu-tRNA(Glu)LARS L-IlePPiDARS EEF1E1 aminoacyl-tRNAsynthetasemultienzyme complexFARSA tRNA(Asp)ATPATPPPiAIMP2 tRNA(Cys)EPRS PPiAIMP2 RARS Gly-tRNA(Gly)AIMP1(1-312) SARS2 PPiEPRS Lys-tRNA(Lys)AMPAMPEARS2aminoacyl-tRNAsynthetasemultienzyme complexPiAMPGARS AMPKARS aminoacyl-tRNAsynthetasemultienzyme complextRNA(Gln)PPA1 L-CysEEF1E1 tRNA(Ile)QARS PPiRARS MARS AMPKARS ATPtRNA(Tyr)tRNA(Arg)TARS Ala-tRNA(Ala)PPiPPiATPMARS tRNA(Pro)GlyEPRS AMPAMPTARS dimerAMPEEF1E1 LARS Thr-tRNA(Thr)AMPPPiEEF1E1 tRNA(Val)L-ProIle-tRNA(Ile)Trp-tRNA(Trp)AIMP1(1-312) PPA1 dimerPPiYARS2 dimerQARS ATPtRNA(Pro)AMPAIMP2 Asn-tRNA(Asn)SARS2 dimerTyr-tRNA(Tyr)Glu-tRNA(Glu)L-ThrPPiAMPFARS2(1-?)ATPL-MetIARS ATPAMPATPL-LysRARS aminoacyl-tRNAsynthetasemultienzyme complexAMPAARS2EEF1E1 MARS AIMP2 MARS L-LysMARS PPitRNA(Thr)Ser-tRNA(Ser)PPiPPiAIMP1(1-312) RARS L-GluATPAMPSer-tRNA(Ser)AMPEEF1E1 tRNA(Ala)AMPL-AspAMPL-ValPPiLARS2(1-?)LARS aminoacyl-tRNAsynthetasemultienzyme complexAMPAsp-tRNA(Asp)tRNA(Leu)QARS ATPL-IleYARS2 L-LeuPPiATPPPiPPiAIMP2 tRNA(Gln)DARS 19191919191919194, 4319


Description

tRNA synthetases catalyze the ligation of tRNAs to their cognate amino acids in an ATP-dependent manner. The reaction proceeds in two steps. First, amino acid and ATP form an aminoacyl adenylate molecule, releasing pyrophosphate. The aminoacyl adenylate remains associated with the synthetase enzyme where, in the second step it reacts with tRNA to form aminoacyl tRNA and AMP. The rapid hydrolysis of pyrophosphate makes these reactions essentially irreversible under physiological conditions (Fersht and Kaethner 1976a). Specificity of the tRNA charging reactions is achieved both by specific recognition of amino acid and tRNA substrates by the synthetase, and by an editing process in which incorrect aminoacyl adenylate molecules (e.g., valyl adenylate associated with isoleucyl tRNA synthetase) are hydrolyzed rather than conjugated to tRNAs in the second step of the reaction (Baldwin and Berg 1966a,b; Fersht and Kaethner 1976b). The tRNA synthetases can be divided into two structural classes based on conserved amino acid sequence features (Burnbaum and Schimmel 1991).

A single synthetase mediates the charging of all of the tRNA species specific for any one amino acid but, with three exceptions, glycine, lysine, and glutamine, the synthetase that catalyzes aminoacylation of mitochondrial tRNAs is encoded by a different gene than the one that acts on mitochondrial tRNAs. Both mitochondrial and cytosolic tRNA synthetase enzymes are encoded by genes in the nuclear genome.<p>A number of tRNA synthetases are known to have functions distinct from tRNA charging (reviewed by Park et al. 2005). Additionally, mutations in several of the tRNA synthetases, often affecting protein domains that are dispensable in vitro for aminoacyl tRNA synthesis, are associated with a diverse array of neurological and other diseases (Antonellis and Green 2008; Park et al. 2008). These findings raise interest into the role of these enzymes in human development and disease.<p> View original pathway at:Reactome.</div>

Try the New WikiPathways

View approved pathways at the new wikipathways.org.

Quality Tags

Ontology Terms

 

Bibliography

View all...
  1. Vilalta A, Donovan D, Wood L, Vogeli G, Yang DC.; ''Cloning, sequencing and expression of a cDNA encoding mammalian valyl-tRNA synthetase.''; PubMed Europe PMC Scholia
  2. Kaminska M, Shalak V, Mirande M.; ''The appended C-domain of human methionyl-tRNA synthetase has a tRNA-sequestering function.''; PubMed Europe PMC Scholia
  3. Spencer AC, Heck A, Takeuchi N, Watanabe K, Spremulli LL.; ''Characterization of the human mitochondrial methionyl-tRNA synthetase.''; PubMed Europe PMC Scholia
  4. Burbaum JJ, Schimmel P.; ''Structural relationships and the classification of aminoacyl-tRNA synthetases.''; PubMed Europe PMC Scholia
  5. O'Hanlon TP, Raben N, Miller FW.; ''A novel gene oriented in a head-to-head configuration with the human histidyl-tRNA synthetase (HRS) gene encodes an mRNA that predicts a polypeptide homologous to HRS.''; PubMed Europe PMC Scholia
  6. Bullard JM, Cai YC, Spremulli LL.; ''Expression and characterization of the human mitochondrial leucyl-tRNA synthetase.''; PubMed Europe PMC Scholia
  7. Lamour V, Quevillon S, Diriong S, N'Guyen VC, Lipinski M, Mirande M.; ''Evolution of the Glx-tRNA synthetase family: the glutaminyl enzyme as a case of horizontal gene transfer.''; PubMed Europe PMC Scholia
  8. Park SG, Schimmel P, Kim S.; ''Aminoacyl tRNA synthetases and their connections to disease.''; PubMed Europe PMC Scholia
  9. Jordanova A, Irobi J, Thomas FP, Van Dijck P, Meerschaert K, Dewil M, Dierick I, Jacobs A, De Vriendt E, Guergueltcheva V, Rao CV, Tournev I, Gondim FA, D'Hooghe M, Van Gerwen V, Callaerts P, Van Den Bosch L, Timmermans JP, Robberecht W, Gettemans J, Thevelein JM, De Jonghe P, Kremensky I, Timmerman V.; ''Disrupted function and axonal distribution of mutant tyrosyl-tRNA synthetase in dominant intermediate Charcot-Marie-Tooth neuropathy.''; PubMed Europe PMC Scholia
  10. Shiba K, Ripmaster T, Suzuki N, Nichols R, Plotz P, Noda T, Schimmel P.; ''Human alanyl-tRNA synthetase: conservation in evolution of catalytic core and microhelix recognition.''; PubMed Europe PMC Scholia
  11. Edvardson S, Shaag A, Kolesnikova O, Gomori JM, Tarassov I, Einbinder T, Saada A, Elpeleg O.; ''Deleterious mutation in the mitochondrial arginyl-transfer RNA synthetase gene is associated with pontocerebellar hypoplasia.''; PubMed Europe PMC Scholia
  12. Curbo S, Lagier-Tourenne C, Carrozzo R, Palenzuela L, Lucioli S, Hirano M, Santorelli F, Arenas J, Karlsson A, Johansson M.; ''Human mitochondrial pyrophosphatase: cDNA cloning and analysis of the gene in patients with mtDNA depletion syndromes.''; PubMed Europe PMC Scholia
  13. Fersht AR, Kaethner MM.; ''Mechanism of aminoacylation of tRNA. Proof of the aminoacyl adenylate pathway for the isoleucyl- and tyrosyl-tRNA synthetases from Escherichia coli K12.''; PubMed Europe PMC Scholia
  14. Fisher RA, Turner BM, Dorkin HL, Harris H.; ''Studies on human erythrocyte inorganic pyrophosphatase.''; PubMed Europe PMC Scholia
  15. Scheper GC, van der Klok T, van Andel RJ, van Berkel CG, Sissler M, Smet J, Muravina TI, Serkov SV, Uziel G, Bugiani M, Schiffmann R, Krägeloh-Mann I, Smeitink JA, Florentz C, Van Coster R, Pronk JC, van der Knaap MS.; ''Mitochondrial aspartyl-tRNA synthetase deficiency causes leukoencephalopathy with brain stem and spinal cord involvement and lactate elevation.''; PubMed Europe PMC Scholia
  16. Wolfe CL, Warrington JA, Davis S, Green S, Norcum MT.; ''Isolation and characterization of human nuclear and cytosolic multisynthetase complexes and the intracellular distribution of p43/EMAPII.''; PubMed Europe PMC Scholia
  17. Degoul F, Brulé H, Cepanec C, Helm M, Marsac C, Leroux J, Giegé R, Florentz C.; ''Isoleucylation properties of native human mitochondrial tRNAIle and tRNAIle transcripts. Implications for cardiomyopathy-related point mutations (4269, 4317) in the tRNAIle gene.''; PubMed Europe PMC Scholia
  18. Jorgensen R, Søgaard TM, Rossing AB, Martensen PM, Justesen J.; ''Identification and characterization of human mitochondrial tryptophanyl-tRNA synthetase.''; PubMed Europe PMC Scholia
  19. Baldwin AN, Berg P.; ''Transfer ribonucleic acid-induced hydrolysis of valyladenylate bound to isoleucyl ribonucleic acid synthetase.''; PubMed Europe PMC Scholia
  20. Antonellis A, Ellsworth RE, Sambuughin N, Puls I, Abel A, Lee-Lin SQ, Jordanova A, Kremensky I, Christodoulou K, Middleton LT, Sivakumar K, Ionasescu V, Funalot B, Vance JM, Goldfarb LG, Fischbeck KH, Green ED.; ''Glycyl tRNA synthetase mutations in Charcot-Marie-Tooth disease type 2D and distal spinal muscular atrophy type V.''; PubMed Europe PMC Scholia
  21. Park SG, Ewalt KL, Kim S.; ''Functional expansion of aminoacyl-tRNA synthetases and their interacting factors: new perspectives on housekeepers.''; PubMed Europe PMC Scholia
  22. Ling C, Yao YN, Zheng YG, Wei H, Wang L, Wu XF, Wang ED.; ''The C-terminal appended domain of human cytosolic leucyl-tRNA synthetase is indispensable in its interaction with arginyl-tRNA synthetase in the multi-tRNA synthetase complex.''; PubMed Europe PMC Scholia
  23. Moor N, Linshiz G, Safro M.; ''Cloning and expression of human phenylalanyl-tRNA synthetase in Escherichia coli: comparative study of purified recombinant enzymes.''; PubMed Europe PMC Scholia
  24. Yang XL, Otero FJ, Skene RJ, McRee DE, Schimmel P, Ribas de Pouplana L.; ''Crystal structures that suggest late development of genetic code components for differentiating aromatic side chains.''; PubMed Europe PMC Scholia
  25. Beaulande M, Tarbouriech N, Härtlein M.; ''Human cytosolic asparaginyl-tRNA synthetase: cDNA sequence, functional expression in Escherichia coli and characterization as human autoantigen.''; PubMed Europe PMC Scholia
  26. Vincent C, Tarbouriech N, Härtlein M.; ''Genomic organization, cDNA sequence, bacterial expression, and purification of human seryl-tRNA synthase.''; PubMed Europe PMC Scholia
  27. Pan F, Lee HH, Pai SH, Lo KY.; ''Purification and subunit structure studies of human placental threonyl-tRNA synthetase.''; PubMed Europe PMC Scholia
  28. Yu Y, Liu Y, Shen N, Xu X, Xu F, Jia J, Jin Y, Arnold E, Ding J.; ''Crystal structure of human tryptophanyl-tRNA synthetase catalytic fragment: insights into substrate recognition, tRNA binding, and angiogenesis activity.''; PubMed Europe PMC Scholia
  29. Escalante C, Yang DC.; ''Expression of human aspartyl-tRNA synthetase in Escherichia coli. Functional analysis of the N-terminal putative amphiphilic helix.''; PubMed Europe PMC Scholia
  30. Bange FC, Flohr T, Buwitt U, Böttger EC.; ''An interferon-induced protein with release factor activity is a tryptophanyl-tRNA synthetase.''; PubMed Europe PMC Scholia
  31. Shiba K, Suzuki N, Shigesada K, Namba Y, Schimmel P, Noda T.; ''Human cytoplasmic isoleucyl-tRNA synthetase: selective divergence of the anticodon-binding domain and acquisition of a new structural unit.''; PubMed Europe PMC Scholia
  32. Pynes GD, Younathan ES.; ''Purification and some properties of inorganic pyrophosphatase from human erythrocytes.''; PubMed Europe PMC Scholia
  33. Rubin BY, Anderson SL, Xing L, Powell RJ, Tate WP.; ''Interferon induces tryptophanyl-tRNA synthetase expression in human fibroblasts.''; PubMed Europe PMC Scholia
  34. Davidson E, Caffarella J, Vitseva O, Hou YM, King MP.; ''Isolation of two cDNAs encoding functional human cytoplasmic cysteinyl-tRNA synthetase.''; PubMed Europe PMC Scholia
  35. Baldwin AN, Berg P.; ''Purification and properties of isoleucyl ribonucleic acid synthetase from Escherichia coli.''; PubMed Europe PMC Scholia
  36. Thuillier L.; ''Purification and kinetic properties of human erythrocyte Mg2+-dependent inorganic pyrophosphatase.''; PubMed Europe PMC Scholia
  37. Lee JW, Beebe K, Nangle LA, Jang J, Longo-Guess CM, Cook SA, Davisson MT, Sundberg JP, Schimmel P, Ackerman SL.; ''Editing-defective tRNA synthetase causes protein misfolding and neurodegeneration.''; PubMed Europe PMC Scholia
  38. Bullard JM, Cai YC, Demeler B, Spremulli LL.; ''Expression and characterization of a human mitochondrial phenylalanyl-tRNA synthetase.''; PubMed Europe PMC Scholia
  39. Kaiser E, Hu B, Becher S, Eberhard D, Schray B, Baack M, Hameister H, Knippers R.; ''The human EPRS locus (formerly the QARS locus): a gene encoding a class I and a class II aminoacyl-tRNA synthetase.''; PubMed Europe PMC Scholia
  40. Bonnefond L, Fender A, Rudinger-Thirion J, Giegé R, Florentz C, Sissler M.; ''Toward the full set of human mitochondrial aminoacyl-tRNA synthetases: characterization of AspRS and TyrRS.''; PubMed Europe PMC Scholia
  41. Fersht AR, Kaethner MM.; ''Enzyme hyperspecificity. Rejection of threonine by the valyl-tRNA synthetase by misacylation and hydrolytic editing.''; PubMed Europe PMC Scholia
  42. Yokogawa T, Shimada N, Takeuchi N, Benkowski L, Suzuki T, Omori A, Ueda T, Nishikawa K, Spremulli LL, Watanabe K.; ''Characterization and tRNA recognition of mammalian mitochondrial seryl-tRNA synthetase.''; PubMed Europe PMC Scholia
  43. Sang Lee J, Gyu Park S, Park H, Seol W, Lee S, Kim S.; ''Interaction network of human aminoacyl-tRNA synthetases and subunits of elongation factor 1 complex.''; PubMed Europe PMC Scholia
  44. Shiba K, Stello T, Motegi H, Noda T, Musier-Forsyth K, Schimmel P.; ''Human lysyl-tRNA synthetase accepts nucleotide 73 variants and rescues Escherichia coli double-defective mutant.''; PubMed Europe PMC Scholia
  45. Antonellis A, Green ED.; ''The role of aminoacyl-tRNA synthetases in genetic diseases.''; PubMed Europe PMC Scholia

History

View all...
CompareRevisionActionTimeUserComment
116664view12:24, 9 May 2021EweitzModified title
114669view16:14, 25 January 2021ReactomeTeamReactome version 75
113116view11:18, 2 November 2020ReactomeTeamReactome version 74
112350view15:27, 9 October 2020ReactomeTeamReactome version 73
101251view11:14, 1 November 2018ReactomeTeamreactome version 66
100790view20:42, 31 October 2018ReactomeTeamreactome version 65
100332view19:19, 31 October 2018ReactomeTeamreactome version 64
99877view16:02, 31 October 2018ReactomeTeamreactome version 63
99434view14:36, 31 October 2018ReactomeTeamreactome version 62 (2nd attempt)
93779view13:35, 16 August 2017ReactomeTeamreactome version 61
93309view11:20, 9 August 2017ReactomeTeamreactome version 61
86391view09:17, 11 July 2016ReactomeTeamreactome version 56
83310view10:45, 18 November 2015ReactomeTeamVersion54
81449view12:59, 21 August 2015ReactomeTeamVersion53
76923view08:19, 17 July 2014ReactomeTeamFixed remaining interactions
76628view12:00, 16 July 2014ReactomeTeamFixed remaining interactions
75959view10:01, 11 June 2014ReactomeTeamRe-fixing comment source
75661view10:56, 10 June 2014ReactomeTeamReactome 48 Update
75016view13:53, 8 May 2014AnweshaFixing comment source for displaying WikiPathways description
74660view08:43, 30 April 2014ReactomeTeamReactome46
74093view12:42, 29 March 2014EgonwFixed the Uniprot-TrEMBL data sources.
45057view19:53, 6 October 2011KhanspersOntology Term : 'translation pathway' added !
42153view22:00, 4 March 2011MaintBotAutomatic update
39964view05:58, 21 January 2011MaintBotNew pathway

External references

DataNodes

View all...
NameTypeDatabase referenceComment
AARS2ProteinQ5JTZ9 (Uniprot-TrEMBL)
AARSProteinP49588 (Uniprot-TrEMBL)
AIMP1(1-312) ProteinQ12904 (Uniprot-TrEMBL)
AIMP2 ProteinQ13155 (Uniprot-TrEMBL)
AMPMetaboliteCHEBI:16027 (ChEBI)
ATPMetaboliteCHEBI:15422 (ChEBI)
Ala-tRNA(Ala)MetaboliteR-HSA-379700 (Reactome)
Ala-tRNA(Ala)MetaboliteR-HSA-379730 (Reactome)
Arg-tRNA(Arg)MetaboliteR-HSA-379708 (Reactome)
Arg-tRNA(Arg)MetaboliteR-HSA-379720 (Reactome)
Asn-tRNA(Asn)MetaboliteR-HSA-379718 (Reactome)
Asn-tRNA(Asn)MetaboliteR-HSA-379728 (Reactome)
Asp-tRNA(Asp)MetaboliteR-HSA-379698 (Reactome)
Asp-tRNA(Asp)MetaboliteR-HSA-379707 (Reactome)
CARS ProteinP49589 (Uniprot-TrEMBL)
CARS dimerComplexR-HSA-379858 (Reactome)
CARS2(1-?)ProteinQ9HA77 (Uniprot-TrEMBL)
Cys-tRNA(Cys)MetaboliteR-HSA-379713 (Reactome)
Cys-tRNA(Cys)MetaboliteR-HSA-379719 (Reactome)
DARS ProteinP14868 (Uniprot-TrEMBL)
DARS2 ProteinQ6PI48 (Uniprot-TrEMBL)
DARS2 dimerComplexR-HSA-379573 (Reactome)
EARS2ProteinQ5JPH6 (Uniprot-TrEMBL)
EEF1E1 ProteinO43324 (Uniprot-TrEMBL)
EPRS ProteinP07814 (Uniprot-TrEMBL)
FARS A2B2 tetramerComplexR-HSA-379637 (Reactome)
FARS2(1-?)ProteinO95363 (Uniprot-TrEMBL)
FARSA ProteinQ9Y285 (Uniprot-TrEMBL)
FARSB ProteinQ9NSD9 (Uniprot-TrEMBL)
GARS ProteinP41250 (Uniprot-TrEMBL)
GARS dimerComplexR-HSA-379593 (Reactome)
GARS dimerComplexR-HSA-379600 (Reactome)
GARS(55-739) ProteinP41250 (Uniprot-TrEMBL)
Gln-tRNA(Gln)MetaboliteR-HSA-379753 (Reactome)
Gln-tRNA(Gln)MetaboliteR-HSA-379772 (Reactome)
Glu-tRNA(Glu)MetaboliteR-HSA-379744 (Reactome)
Glu-tRNA(Glu)MetaboliteR-HSA-379751 (Reactome)
GluMetaboliteCHEBI:16015 (ChEBI)
Gly-tRNA(Gly)MetaboliteR-HSA-379781 (Reactome)
Gly-tRNA(Gly)MetaboliteR-HSA-379784 (Reactome)
GlyMetaboliteCHEBI:15428 (ChEBI)
H2OMetaboliteCHEBI:15377 (ChEBI)
HARS2ProteinP49590 (Uniprot-TrEMBL)
HARSProteinP12081 (Uniprot-TrEMBL)
His-tRNA(His)MetaboliteR-HSA-379764 (Reactome)
His-tRNA(His)MetaboliteR-HSA-379786 (Reactome)
IARS ProteinP41252 (Uniprot-TrEMBL)
IARS2ProteinQ9NSE4 (Uniprot-TrEMBL)
Ile-tRNA(Ile)MetaboliteR-HSA-379769 (Reactome)
Ile-tRNA(Ile)MetaboliteR-HSA-379787 (Reactome)
KARS ProteinQ15046 (Uniprot-TrEMBL)
KARS dimerComplexR-HSA-379687 (Reactome)
L-AlaMetaboliteCHEBI:16977 (ChEBI)
L-ArgMetaboliteCHEBI:16467 (ChEBI)
L-AsnMetaboliteCHEBI:17196 (ChEBI)
L-AspMetaboliteCHEBI:17053 (ChEBI)
L-CysMetaboliteCHEBI:17561 (ChEBI)
L-GlnMetaboliteCHEBI:18050 (ChEBI)
L-GluMetaboliteCHEBI:16015 (ChEBI)
L-HisMetaboliteCHEBI:15971 (ChEBI)
L-IleMetaboliteCHEBI:17191 (ChEBI)
L-LeuMetaboliteCHEBI:15603 (ChEBI)
L-LysMetaboliteCHEBI:18019 (ChEBI)
L-MetMetaboliteCHEBI:16643 (ChEBI)
L-PheMetaboliteCHEBI:17295 (ChEBI)
L-ProMetaboliteCHEBI:17203 (ChEBI)
L-SerMetaboliteCHEBI:17115 (ChEBI)
L-ThrMetaboliteCHEBI:16857 (ChEBI)
L-TrpMetaboliteCHEBI:16828 (ChEBI)
L-TyrMetaboliteCHEBI:17895 (ChEBI)
L-ValMetaboliteCHEBI:16414 (ChEBI)
LARS ProteinQ9P2J5 (Uniprot-TrEMBL)
LARS2(1-?)ProteinQ15031 (Uniprot-TrEMBL)
Leu-tRNA(Leu)MetaboliteR-HSA-379757 (Reactome)
Leu-tRNA(Leu)MetaboliteR-HSA-379773 (Reactome)
Lys-tRNA(Lys)MetaboliteR-HSA-379736 (Reactome)
Lys-tRNA(Lys)MetaboliteR-HSA-379795 (Reactome)
MARS ProteinP56192 (Uniprot-TrEMBL)
MARS2ProteinQ96GW9 (Uniprot-TrEMBL)
Met-tRNA(Met)MetaboliteR-HSA-379780 (Reactome)
Met-tRNA(Met)MetaboliteR-HSA-379794 (Reactome)
Mg2+MetaboliteCHEBI:18420 (ChEBI)
NARS2ProteinQ96I59 (Uniprot-TrEMBL)
NARSProteinO43776 (Uniprot-TrEMBL)
PARS2ProteinQ7L3T8 (Uniprot-TrEMBL)
PPA1 ProteinQ15181 (Uniprot-TrEMBL)
PPA1 dimerComplexR-HSA-71726 (Reactome)
PPA2 ProteinQ9H2U2 (Uniprot-TrEMBL)
PPA2 dimerComplexR-HSA-449923 (Reactome)
PPiMetaboliteCHEBI:29888 (ChEBI)
Phe-tRNA(Phe)MetaboliteR-HSA-379789 (Reactome)
Phe-tRNA(Phe)MetaboliteR-HSA-379792 (Reactome)
PiMetaboliteCHEBI:18367 (ChEBI)
Pro-tRNA(Pro)MetaboliteR-HSA-379745 (Reactome)
Pro-tRNA(Pro)MetaboliteR-HSA-379746 (Reactome)
QARS ProteinP47897 (Uniprot-TrEMBL)
QARSProteinP47897 (Uniprot-TrEMBL)
RARS ProteinP54136 (Uniprot-TrEMBL)
RARS2ProteinQ5T160 (Uniprot-TrEMBL)
SARS ProteinP49591 (Uniprot-TrEMBL)
SARS dimerComplexR-HSA-379557 (Reactome)
SARS2 ProteinQ9NP81 (Uniprot-TrEMBL)
SARS2 dimerComplexR-HSA-379672 (Reactome)
Ser-tRNA(Ser)MetaboliteR-HSA-379738 (Reactome)
Ser-tRNA(Ser)MetaboliteR-HSA-379777 (Reactome)
SerMetaboliteCHEBI:17115 (ChEBI)
TARS ProteinP26639 (Uniprot-TrEMBL)
TARS dimerComplexR-HSA-379624 (Reactome)
TARS2(1-?)ProteinQ9BW92 (Uniprot-TrEMBL)
Thr-tRNA(Thr)MetaboliteR-HSA-379779 (Reactome)
Thr-tRNA(Thr)MetaboliteR-HSA-379783 (Reactome)
Trp-tRNA(Trp)MetaboliteR-HSA-379759 (Reactome)
Trp-tRNA(Trp)MetaboliteR-HSA-379765 (Reactome)
Tyr-tRNA(Tyr)MetaboliteR-HSA-379755 (Reactome)
Tyr-tRNA(Tyr)MetaboliteR-HSA-379785 (Reactome)
VARS2ProteinQ5ST30 (Uniprot-TrEMBL)
VARSProteinP26640 (Uniprot-TrEMBL)
Val-tRNA(Val)MetaboliteR-HSA-379782 (Reactome)
Val-tRNA(Val)MetaboliteR-HSA-379790 (Reactome)
WARS ProteinP23381 (Uniprot-TrEMBL)
WARS dimerComplexR-HSA-379684 (Reactome)
WARS2ProteinQ9UGM6 (Uniprot-TrEMBL)
YARS ProteinP54577 (Uniprot-TrEMBL)
YARS dimerComplexR-HSA-379566 (Reactome)
YARS2 ProteinQ9Y2Z4 (Uniprot-TrEMBL)
YARS2 dimerComplexR-HSA-379656 (Reactome)
aminoacyl-tRNA

synthetase

multienzyme complex		ComplexR-HSA-379688 (Reactome)
tRNA(Ala)MetaboliteR-HSA-379729 (Reactome)
tRNA(Ala)MetaboliteR-HSA-379731 (Reactome)
tRNA(Arg)MetaboliteR-HSA-379704 (Reactome)
tRNA(Arg)MetaboliteR-HSA-379727 (Reactome)
tRNA(Asn)MetaboliteR-HSA-379699 (Reactome)
tRNA(Asn)MetaboliteR-HSA-379722 (Reactome)
tRNA(Asp)MetaboliteR-HSA-379711 (Reactome)
tRNA(Asp)MetaboliteR-HSA-379715 (Reactome)
tRNA(Cys)MetaboliteR-HSA-379714 (Reactome)
tRNA(Cys)MetaboliteR-HSA-379725 (Reactome)
tRNA(Gln)MetaboliteR-HSA-379740 (Reactome)
tRNA(Gln)MetaboliteR-HSA-379748 (Reactome)
tRNA(Glu)MetaboliteR-HSA-379754 (Reactome)
tRNA(Glu)MetaboliteR-HSA-379778 (Reactome)
tRNA(Gly)MetaboliteR-HSA-379766 (Reactome)
tRNA(Gly)MetaboliteR-HSA-379770 (Reactome)
tRNA(His)MetaboliteR-HSA-379752 (Reactome)
tRNA(His)MetaboliteR-HSA-379758 (Reactome)
tRNA(Ile)MetaboliteR-HSA-379739 (Reactome)
tRNA(Ile)MetaboliteR-HSA-379750 (Reactome)
tRNA(Leu)MetaboliteR-HSA-379771 (Reactome)
tRNA(Leu)MetaboliteR-HSA-379788 (Reactome)
tRNA(Lys)MetaboliteR-HSA-379747 (Reactome)
tRNA(Lys)MetaboliteR-HSA-379776 (Reactome)
tRNA(Met)MetaboliteR-HSA-379741 (Reactome)
tRNA(Met)MetaboliteR-HSA-379742 (Reactome)
tRNA(Phe)MetaboliteR-HSA-379760 (Reactome)
tRNA(Phe)MetaboliteR-HSA-379767 (Reactome)
tRNA(Pro)MetaboliteR-HSA-379768 (Reactome)
tRNA(Pro)MetaboliteR-HSA-379775 (Reactome)
tRNA(Ser)MetaboliteR-HSA-379761 (Reactome)
tRNA(Ser)MetaboliteR-HSA-379762 (Reactome)
tRNA(Thr)MetaboliteR-HSA-379749 (Reactome)
tRNA(Thr)MetaboliteR-HSA-379791 (Reactome)
tRNA(Trp)MetaboliteR-HSA-379763 (Reactome)
tRNA(Trp)MetaboliteR-HSA-379774 (Reactome)
tRNA(Tyr)MetaboliteR-HSA-379743 (Reactome)
tRNA(Tyr)MetaboliteR-HSA-379756 (Reactome)
tRNA(Val)MetaboliteR-HSA-379735 (Reactome)
tRNA(Val)MetaboliteR-HSA-379793 (Reactome)

Annotated Interactions

View all...
SourceTargetTypeDatabase referenceComment
AARS2mim-catalysisR-HSA-380177 (Reactome)
AARSmim-catalysisR-HSA-379864 (Reactome)
AMPArrowR-HSA-379844 (Reactome)
AMPArrowR-HSA-379848 (Reactome)
AMPArrowR-HSA-379861 (Reactome)
AMPArrowR-HSA-379864 (Reactome)
AMPArrowR-HSA-379865 (Reactome)
AMPArrowR-HSA-379867 (Reactome)
AMPArrowR-HSA-379887 (Reactome)
AMPArrowR-HSA-379893 (Reactome)
AMPArrowR-HSA-379974 (Reactome)
AMPArrowR-HSA-379977 (Reactome)
AMPArrowR-HSA-379980 (Reactome)
AMPArrowR-HSA-379982 (Reactome)
AMPArrowR-HSA-379992 (Reactome)
AMPArrowR-HSA-379993 (Reactome)
AMPArrowR-HSA-379994 (Reactome)
AMPArrowR-HSA-379996 (Reactome)
AMPArrowR-HSA-380002 (Reactome)
AMPArrowR-HSA-380008 (Reactome)
AMPArrowR-HSA-380042 (Reactome)
AMPArrowR-HSA-380048 (Reactome)
AMPArrowR-HSA-380157 (Reactome)
AMPArrowR-HSA-380158 (Reactome)
AMPArrowR-HSA-380170 (Reactome)
AMPArrowR-HSA-380176 (Reactome)
AMPArrowR-HSA-380177 (Reactome)
AMPArrowR-HSA-380198 (Reactome)
AMPArrowR-HSA-380199 (Reactome)
AMPArrowR-HSA-380200 (Reactome)
AMPArrowR-HSA-380201 (Reactome)
AMPArrowR-HSA-380203 (Reactome)
AMPArrowR-HSA-380216 (Reactome)
AMPArrowR-HSA-380222 (Reactome)
AMPArrowR-HSA-380224 (Reactome)
AMPArrowR-HSA-380227 (Reactome)
AMPArrowR-HSA-380229 (Reactome)
AMPArrowR-HSA-380233 (Reactome)
AMPArrowR-HSA-380234 (Reactome)
AMPArrowR-HSA-380239 (Reactome)
AMPArrowR-HSA-380240 (Reactome)
AMPArrowR-HSA-380241 (Reactome)
ATPR-HSA-379844 (Reactome)
ATPR-HSA-379848 (Reactome)
ATPR-HSA-379861 (Reactome)
ATPR-HSA-379864 (Reactome)
ATPR-HSA-379865 (Reactome)
ATPR-HSA-379867 (Reactome)
ATPR-HSA-379887 (Reactome)
ATPR-HSA-379893 (Reactome)
ATPR-HSA-379974 (Reactome)
ATPR-HSA-379977 (Reactome)
ATPR-HSA-379980 (Reactome)
ATPR-HSA-379982 (Reactome)
ATPR-HSA-379992 (Reactome)
ATPR-HSA-379993 (Reactome)
ATPR-HSA-379994 (Reactome)
ATPR-HSA-379996 (Reactome)
ATPR-HSA-380002 (Reactome)
ATPR-HSA-380008 (Reactome)
ATPR-HSA-380042 (Reactome)
ATPR-HSA-380048 (Reactome)
ATPR-HSA-380157 (Reactome)
ATPR-HSA-380158 (Reactome)
ATPR-HSA-380170 (Reactome)
ATPR-HSA-380176 (Reactome)
ATPR-HSA-380177 (Reactome)
ATPR-HSA-380198 (Reactome)
ATPR-HSA-380199 (Reactome)
ATPR-HSA-380200 (Reactome)
ATPR-HSA-380201 (Reactome)
ATPR-HSA-380203 (Reactome)
ATPR-HSA-380216 (Reactome)
ATPR-HSA-380222 (Reactome)
ATPR-HSA-380224 (Reactome)
ATPR-HSA-380227 (Reactome)
ATPR-HSA-380229 (Reactome)
ATPR-HSA-380233 (Reactome)
ATPR-HSA-380234 (Reactome)
ATPR-HSA-380239 (Reactome)
ATPR-HSA-380240 (Reactome)
ATPR-HSA-380241 (Reactome)
Ala-tRNA(Ala)ArrowR-HSA-379864 (Reactome)
Ala-tRNA(Ala)ArrowR-HSA-380177 (Reactome)
Arg-tRNA(Arg)ArrowR-HSA-379993 (Reactome)
Arg-tRNA(Arg)ArrowR-HSA-380224 (Reactome)
Asn-tRNA(Asn)ArrowR-HSA-379996 (Reactome)
Asn-tRNA(Asn)ArrowR-HSA-380227 (Reactome)
Asp-tRNA(Asp)ArrowR-HSA-379867 (Reactome)
Asp-tRNA(Asp)ArrowR-HSA-380229 (Reactome)
CARS dimermim-catalysisR-HSA-379887 (Reactome)
CARS2(1-?)mim-catalysisR-HSA-380158 (Reactome)
Cys-tRNA(Cys)ArrowR-HSA-379887 (Reactome)
Cys-tRNA(Cys)ArrowR-HSA-380158 (Reactome)
DARS2 dimermim-catalysisR-HSA-380229 (Reactome)
EARS2mim-catalysisR-HSA-380216 (Reactome)
FARS A2B2 tetramermim-catalysisR-HSA-379848 (Reactome)
FARS2(1-?)mim-catalysisR-HSA-380203 (Reactome)
GARS dimermim-catalysisR-HSA-380048 (Reactome)
GARS dimermim-catalysisR-HSA-380240 (Reactome)
Gln-tRNA(Gln)ArrowR-HSA-379982 (Reactome)
Gln-tRNA(Gln)ArrowR-HSA-380241 (Reactome)
Glu-tRNA(Glu)ArrowR-HSA-379861 (Reactome)
Glu-tRNA(Glu)ArrowR-HSA-380216 (Reactome)
GluR-HSA-380216 (Reactome)
Gly-tRNA(Gly)ArrowR-HSA-380048 (Reactome)
Gly-tRNA(Gly)ArrowR-HSA-380240 (Reactome)
GlyR-HSA-380048 (Reactome)
GlyR-HSA-380240 (Reactome)
H2OR-HSA-449937 (Reactome)
H2OR-HSA-71732 (Reactome)
HARS2mim-catalysisR-HSA-380234 (Reactome)
HARSmim-catalysisR-HSA-379844 (Reactome)
His-tRNA(His)ArrowR-HSA-379844 (Reactome)
His-tRNA(His)ArrowR-HSA-380234 (Reactome)
IARS2mim-catalysisR-HSA-380176 (Reactome)
Ile-tRNA(Ile)ArrowR-HSA-379893 (Reactome)
Ile-tRNA(Ile)ArrowR-HSA-380176 (Reactome)
KARS dimermim-catalysisR-HSA-380233 (Reactome)
L-AlaR-HSA-379864 (Reactome)
L-AlaR-HSA-380177 (Reactome)
L-ArgR-HSA-379993 (Reactome)
L-ArgR-HSA-380224 (Reactome)
L-AsnR-HSA-379996 (Reactome)
L-AsnR-HSA-380227 (Reactome)
L-AspR-HSA-379867 (Reactome)
L-AspR-HSA-380229 (Reactome)
L-CysR-HSA-379887 (Reactome)
L-CysR-HSA-380158 (Reactome)
L-GlnR-HSA-379982 (Reactome)
L-GlnR-HSA-380241 (Reactome)
L-GluR-HSA-379861 (Reactome)
L-HisR-HSA-379844 (Reactome)
L-HisR-HSA-380234 (Reactome)
L-IleR-HSA-379893 (Reactome)
L-IleR-HSA-380176 (Reactome)
L-LeuR-HSA-379974 (Reactome)
L-LeuR-HSA-380200 (Reactome)
L-LysR-HSA-380008 (Reactome)
L-LysR-HSA-380233 (Reactome)
L-MetR-HSA-379994 (Reactome)
L-MetR-HSA-380157 (Reactome)
L-PheR-HSA-379848 (Reactome)
L-PheR-HSA-380203 (Reactome)
L-ProR-HSA-379865 (Reactome)
L-ProR-HSA-380198 (Reactome)
L-SerR-HSA-380239 (Reactome)
L-ThrR-HSA-380002 (Reactome)
L-ThrR-HSA-380201 (Reactome)
L-TrpR-HSA-379977 (Reactome)
L-TrpR-HSA-380222 (Reactome)
L-TyrR-HSA-379980 (Reactome)
L-TyrR-HSA-380170 (Reactome)
L-ValR-HSA-380042 (Reactome)
L-ValR-HSA-380199 (Reactome)
LARS2(1-?)mim-catalysisR-HSA-380200 (Reactome)
Leu-tRNA(Leu)ArrowR-HSA-379974 (Reactome)
Leu-tRNA(Leu)ArrowR-HSA-380200 (Reactome)
Lys-tRNA(Lys)ArrowR-HSA-380008 (Reactome)
Lys-tRNA(Lys)ArrowR-HSA-380233 (Reactome)
MARS2mim-catalysisR-HSA-380157 (Reactome)
Met-tRNA(Met)ArrowR-HSA-379994 (Reactome)
Met-tRNA(Met)ArrowR-HSA-380157 (Reactome)
Mg2+ArrowR-HSA-449937 (Reactome)
Mg2+ArrowR-HSA-71732 (Reactome)
NARS2mim-catalysisR-HSA-380227 (Reactome)
NARSmim-catalysisR-HSA-379996 (Reactome)
PARS2mim-catalysisR-HSA-380198 (Reactome)
PPA1 dimermim-catalysisR-HSA-71732 (Reactome)
PPA2 dimermim-catalysisR-HSA-449937 (Reactome)
PPiArrowR-HSA-379844 (Reactome)
PPiArrowR-HSA-379848 (Reactome)
PPiArrowR-HSA-379861 (Reactome)
PPiArrowR-HSA-379864 (Reactome)
PPiArrowR-HSA-379865 (Reactome)
PPiArrowR-HSA-379867 (Reactome)
PPiArrowR-HSA-379887 (Reactome)
PPiArrowR-HSA-379893 (Reactome)
PPiArrowR-HSA-379974 (Reactome)
PPiArrowR-HSA-379977 (Reactome)
PPiArrowR-HSA-379980 (Reactome)
PPiArrowR-HSA-379982 (Reactome)
PPiArrowR-HSA-379992 (Reactome)
PPiArrowR-HSA-379993 (Reactome)
PPiArrowR-HSA-379994 (Reactome)
PPiArrowR-HSA-379996 (Reactome)
PPiArrowR-HSA-380002 (Reactome)
PPiArrowR-HSA-380008 (Reactome)
PPiArrowR-HSA-380042 (Reactome)
PPiArrowR-HSA-380048 (Reactome)
PPiArrowR-HSA-380157 (Reactome)
PPiArrowR-HSA-380158 (Reactome)
PPiArrowR-HSA-380170 (Reactome)
PPiArrowR-HSA-380176 (Reactome)
PPiArrowR-HSA-380177 (Reactome)
PPiArrowR-HSA-380198 (Reactome)
PPiArrowR-HSA-380199 (Reactome)
PPiArrowR-HSA-380200 (Reactome)
PPiArrowR-HSA-380201 (Reactome)
PPiArrowR-HSA-380203 (Reactome)
PPiArrowR-HSA-380216 (Reactome)
PPiArrowR-HSA-380222 (Reactome)
PPiArrowR-HSA-380224 (Reactome)
PPiArrowR-HSA-380227 (Reactome)
PPiArrowR-HSA-380229 (Reactome)
PPiArrowR-HSA-380233 (Reactome)
PPiArrowR-HSA-380234 (Reactome)
PPiArrowR-HSA-380239 (Reactome)
PPiArrowR-HSA-380240 (Reactome)
PPiArrowR-HSA-380241 (Reactome)
PPiR-HSA-449937 (Reactome)
PPiR-HSA-71732 (Reactome)
Phe-tRNA(Phe)ArrowR-HSA-379848 (Reactome)
Phe-tRNA(Phe)ArrowR-HSA-380203 (Reactome)
PiArrowR-HSA-449937 (Reactome)
PiArrowR-HSA-71732 (Reactome)
Pro-tRNA(Pro)ArrowR-HSA-379865 (Reactome)
Pro-tRNA(Pro)ArrowR-HSA-380198 (Reactome)
QARSmim-catalysisR-HSA-380241 (Reactome)
R-HSA-379844 (Reactome) HARS (cytosolic histidyl tRNA synthetase) catalyzes the reaction of histidine, tRNA(His), and ATP to form His-tRNA(His), AMP, and pyrophosphate (Lee et al. 2002).
R-HSA-379848 (Reactome) FARS (cytosolic phenylalanyl tRNA synthetase) catalyzes the reaction of phenylalanine, tRNA(Phe), and ATP to form Phe-tRNA(Phe), AMP, and pyrophosphate. The enzyme, a class II tRNA synthetase, is a heterotetramer of two alpha and two beta subunits (Moor et al. 2002).
R-HSA-379861 (Reactome) Multifunctional EPRS (cytosolic glutamyl prolyl tRNA synthetase) catalyzes the reaction of glutamate, tRNA(Glu), and ATP to form Glu tRNA(Glu), AMP, and pyrophosphate. The same enzyme also catalyzes the charging of tRNA(Pro) with proline. The enzyme is found in the cell as a component of the mutienzyme aminoacyl tRNA synthetase complex (Kaiser et al. 1994).
R-HSA-379864 (Reactome) AARS (cytosolic alanyl tRNA synthetase) catalyzes the reaction of alanine, tRNA(ala), and ATP to form Ala tRNA(Ala), AMP, and pyrophosphate. The enzyme, a class II tRNA synthetase, is a monomer (Shiba et al. 1995). A mutation in the editing domain of the mouse Aars gene results in misincorporation of non-cognate amino acids into cellular proteins. This is associated with protein misfolding in Purkinje cells and a phenotype consistent with human ataxia (Lee et al. 2006).
R-HSA-379865 (Reactome) Multifunctional EPRS (cytosolic glutamyl-prolyl tRNA synthetase) catalyzes the reaction of proline, tRNA(Pro), and ATP to form Pro-tRNA(Pro), AMP, and pyrophosphate. The same enzyme also catalyzes the charging of tRNA(Glu) with glutamate. The enzyme is found in the cell as a component of the mutienzyme aminoacyl-tRNA synthetase complex (Kaiser et al. 1994).
R-HSA-379867 (Reactome) DARS (cytosolic aspartyl tRNA synthetase) catalyzes the reaction of aspartate, tRNA(Asp), and ATP to form Asp-tRNA(Asp), AMP, and pyrophosphate. The enzyme, a class II tRNA synthetase, is a homodimer found in the cell as a component of the mutienzyme aminoacyl-tRNA synthetase complex (Escalante and Yang 1993).
R-HSA-379887 (Reactome) CARS (cytosolic cysteinyl tRNA synthetase) catalyzes the reaction of cysteine, tRNA(cys), and ATP to form Cys-tRNA(Cys), AMP, and pyrophosphate. The enzyme, a class I tRNA synthetase, is a homomultimer, probably a dimer (Davidson et al. 2001).
R-HSA-379893 (Reactome) IARS (cytosolic aspartyl tRNA synthetase) catalyzes the reaction of isoleucine, tRNA(Ile), and ATP to form Ile-tRNA(Ile), AMP, and pyrophosphate. The enzyme, a class I tRNA synthetase, is found in the cell as a component of the mutienzyme aminoacyl-tRNA synthetase complex (Shiba et al. 1994).
R-HSA-379974 (Reactome) LARS (cytosolic leucyl tRNA synthetase) catalyzes the reaction of leucine, tRNA(Leu), and ATP to form Leu-tRNA(Leu), AMP, and pyrophosphate. The enzyme, a class I tRNA synthetase, is found in the cell as a component of the mutienzyme aminoacyl-tRNA synthetase complex (Ling et al. 2005).
R-HSA-379977 (Reactome) WARS (cytosolic tryptophanyl tRNA synthetase) catalyzes the reaction of tryptophan, tRNA(Trp), and ATP to form Trp-tRNA(Trp), AMP, and pyrophosphate. The enzyme, a class I tRNA synthetase, is a homodimer (Bange et al. 1992; Rubin et al. 1991; Yu et al. 2004).
R-HSA-379980 (Reactome) YARS (cytosolic tyrosyl tRNA synthetase) catalyzes the reaction of tyrosine, tRNA(Tyr), and ATP to form Tyr tRNA(Tyr), AMP, and pyrophosphate. The enzyme, a class I tRNA synthetase, is a homodimer (Yang et al. 2003). Mutations in the YARS gene are associated with dominant-intermediate Charcot-Marie-Tooth disease – a form of peripheral neuropathy characterized by axon and Schwann cell dysfunction (Jordanova et al. 2006).
R-HSA-379982 (Reactome) QARS (cytosolic glutaminyl tRNA synthetase) catalyzes the reaction of glutamine, tRNA(Gln), and ATP to form Gln-tRNA(Gln), AMP, and pyrophosphate. The enzyme, a class I tRNA synthetase, is found in the cell as a component of the mutienzyme aminoacyl-tRNA synthetase complex. The same gene encodes both the cytosolic and mitochondrial QARS enzymes (Lamour et al. 1994).
R-HSA-379992 (Reactome) SARS (cytosolic seryl tRNA synthetase) catalyzes the reaction of serine, tRNA(Ser), and ATP to form Ser-tRNA(Ser), AMP, and pyrophosphate. The enzyme, a class II tRNA synthetase, is a homodimer (Hartlein and Cusack 1995; Vincent et al. 1997).
R-HSA-379993 (Reactome) RARS (cytosolic arginyl tRNA synthetase) catalyzes the reaction of arginine, tRNA(Arg), and ATP to form Arg-tRNA(Arg), AMP, and pyrophosphate. The enzyme, a class I tRNA synthetase, is a monomer found in the cell as a component of the mutienzyme aminoacyl-tRNA synthetase complex (Ling et al. 2005).
R-HSA-379994 (Reactome) MARS (cytosolic methionyl tRNA synthetase) catalyzes the reaction of methionine, tRNA(Met), and ATP to form Met-tRNA(Met), AMP, and pyrophosphate. The enzyme, a class I tRNA synthetase, is a monomer found in the cell as a component of the mutienzyme aminoacyl-tRNA synthetase complex (Kaminska et al. 2001).
R-HSA-379996 (Reactome) NARS (cytosolic asparaginyl tRNA synthetase) catalyzes the reaction of asparagine, tRNA(Asn), and ATP to form Asn-tRNA(Asn), AMP, and pyrophosphate. The enzyme is a class I tRNA synthetase (Beaulande et al. 1998).
R-HSA-380002 (Reactome) TARS (cytosolic threonyl tRNA synthetase) catalyzes the reaction of threonine, tRNA(Thr), and ATP to form Thr-tRNA(Thr), AMP, and pyrophosphate. The enzyme, a class II tRNA synthetase, is a homodimer (Pan et al. 1982).
R-HSA-380008 (Reactome) KARS (cytosolic lysyl tRNA synthetase) catalyzes the reaction of lysine, tRNA(Lys), and ATP to form Lys-tRNA(Lys), AMP, and pyrophosphate. The enzyme, a class II tRNA synthetase, is a homodimer found in the cell as a component of the mutienzyme aminoacyl-tRNA synthetase complex. The same gene encodes both cytosolic and mitochondrial KARS enzymes (Shiba et al. 1997).
R-HSA-380042 (Reactome) VARS (cytosolic valyl tRNA synthetase) catalyzes the reaction of valine, tRNA(Val), and ATP to form Val-tRNA(Val), AMP, and pyrophosphate. The enzyme is a class I tRNA synthetase (Vilalta et al. 1993).
R-HSA-380048 (Reactome) GARS (cytosolic glycyl tRNA synthetase) catalyzes the reaction of glycine, tRNA(Gly), and ATP to form Gly tRNA(Gly), AMP, and pyrophosphate. The enzyme, a class II tRNA synthetase, is a dimer. Cytosolic and mitochondrial glycyl tRNA synthetase enzymes are both encoded by the same gene; the cytosolic protein lacks an aminoterminal 54 residue sequence found in the mitochondrial protein. Mutations in GARS are associated with Charcot Marie Tooth disease and distal spinal muscular atrophy, two diseases characterized by axonal dysfunction (Antonellis et al. 2006)
R-HSA-380157 (Reactome) MARS2 (mitochondrial methionyl tRNA synthetase) catalyzes the reaction of methionine, mitochondrial tRNA(Met), and ATP to form Met-tRNA(Met), AMP, and pyrophosphate. The enzyme, a class I tRNA synthetase, is a monomer (Spencer et al. 2004).
R-HSA-380158 (Reactome) CARS2 (mitochondrial cysteinyl tRNA synthetase) catalyzes the reaction of cysteine, mitochondrial tRNA(Cys), and ATP to form Cys-tRNA(Cys), AMP, and pyrophosphate. The CARS2 gene has been identified by computational analysis of the human genome sequence; its function has been inferred from those of the biochemically characterized mitochondrial aspartyl and tyrosyl tRNA synthetases (Bonnefond et al. 2005).
R-HSA-380170 (Reactome) YARS2 (mitochondrial tyrosyl tRNA synthetase) catalyzes the reaction of tyrosine, mitochondrial tRNA(Tyr), and ATP to form Tyr-tRNA(Tyr), AMP, and pyrophosphate. The enzyme, a class I tRNA synthetase, is a homodimer (Bonnefond et al. 2005).
R-HSA-380176 (Reactome) IARS2 (mitochondrial isoleucyl tRNA synthetase) catalyzes the reaction of isoleucine, mitochondrial tRNA(Ile), and ATP to form Ile-tRNA(Ile), AMP, and pyrophosphate. The enzyme is a class I tRNA synthetase (Degoul et al. 1995).
R-HSA-380177 (Reactome) AARS2 (mitochondrial alanyl tRNA synthetase) catalyzes the reaction of alanine, mitochondrial tRNA(Ala), and ATP to form Ala-tRNA(Ala), AMP, and pyrophosphate. The AARS2 gene has been identified by computational analysis of the human genome sequence; its function has been inferred from those of the biochemically characterized mitochondrial aspartyl and tyrosyl tRNA synthetases (Bonnefond et al. 2005).
R-HSA-380198 (Reactome) PARS2 (mitochondrial prolyl tRNA synthetase) catalyzes the reaction of proline, mitochondrial tRNA(Pro), and ATP to form Pro-tRNA(Pro), AMP, and pyrophosphate. The PARS2 gene has been identified by computational analysis of the human genome sequence; its function has been inferred from those of the biochemically characterized mitochondrial aspartyl and tyrosyl tRNA synthetases (Bonnefond et al. 2005).
R-HSA-380199 (Reactome) VARS2 (mitochondrial valyl tRNA synthetase) catalyzes the reaction of valine, mitochondrial tRNA(val), and ATP to form Val-tRNA(Val), AMP, and pyrophosphate. The VARS2 gene has been identified by computational analysis of the human genome sequence; its function has been inferred from those of the biochemically characterized mitochondrial aspartyl and tyrosyl tRNA synthetases (Bonnefond et al. 2005).
R-HSA-380200 (Reactome) LARS2 (mitochondrial leucyl tRNA synthetase) catalyzes the reaction of leucine, mitochondrial tRNA(Leu), and ATP to form Leu-tRNA(Leu), AMP, and pyrophosphate. The enzyme, a class I tRNA synthetase, is a monomer (Bullard et al. 2000).
R-HSA-380201 (Reactome) TARS2 (mitochondrial threonyl tRNA synthetase) catalyzes the reaction of threonine, mitochondrial tRNA(Thr), and ATP to form Thr-tRNA(Thr), AMP, and pyrophosphate. The TARS2 gene has been identified by computational analysis of the human genome sequence; its function has been inferred from those of the biochemically characterized mitochondrial aspartyl and tyrosyl tRNA synthetases (Bonnefond et al. 2005).
R-HSA-380203 (Reactome) FARS2 (mitochondrial phenylalanyl tRNA synthetase) catalyzes the reaction of phenylalanine, mitochondrial tRNA(Phe), and ATP to form Phe-tRNA(Phe), AMP, and pyrophosphate. The enzyme, a class II tRNA synthetase, is a monomer (Bullard et al. 1999).
R-HSA-380216 (Reactome) EARS2 (mitochondrial glutamyl tRNA synthetase) catalyzes the reaction of glutamate, mitochondrial tRNA(Glu), and ATP to form Glu-tRNA(Glu), AMP, and pyrophosphate. The EARS2 gene has been identified by computational analysis of the human genome sequence; its function has been inferred from those of the biochemically characterized mitochondrial aspartyl and tyrosyl tRNA synthetases (Bonnefond et al. 2005).
R-HSA-380222 (Reactome) WARS2 (mitochondrial tryptophanyl tRNA synthetase) catalyzes the reaction of tryptophan, mitochondrial tRNA(Trp), and ATP to form Trp-tRNA(Trp), AMP, and pyrophosphate. The enzyme is a class I tRNA synthetase (Jorgensen et al. 2000).
R-HSA-380224 (Reactome) RARS2 (mitochondrial arginyl tRNA synthetase) catalyzes the reaction of arginine, mitochondrial tRNA(Arg), and ATP to form Arg tRNA(Arg), AMP, and pyrophosphate. The enzyme is a class I tRNA synthetase. Homozygosity for RARS2 mutations has been associated with pontocerebellar hypoplasia – a disease characterized by a severe reduction in cerebellum and brainstem size (Edvardson et al. 2007).
R-HSA-380227 (Reactome) NARS2 (mitochondrial asparaginyl tRNA synthetase) catalyzes the reaction of asparagine, mitochondrial tRNA(Asn), and ATP to form Asn-tRNA(Asn), AMP, and pyrophosphate. The NARS2 gene has been identified by computational analysis of the human genome sequence; its function has been inferred from those of the biochemically characterized mitochondrial aspartyl and tyrosyl tRNA synthetases (Bonnefond et al. 2005).
R-HSA-380229 (Reactome) DARS2 (mitochondrial aspartyl tRNA synthetase) catalyzes the reaction of aspartate, mitochondrial tRNA(Asp), and ATP to form Asp tRNA(Asp), AMP, and pyrophosphate. The enzyme, a class II tRNA synthetase, is a homodimer (Bonnefond et al. 2005). Homozygosity for DARS2 mutations is associated with leukoencephalopathy with brainstem and spinal cord involvement plus lactate elevation (Scheper et al 2007).
R-HSA-380233 (Reactome) KARS (mitochondrial lysyl tRNA synthetase) catalyzes the reaction of lysine, tRNA(Lys), and ATP to form Lys-tRNA(Lys), AMP, and pyrophosphate. The enzyme, a class II tRNA synthetase, is a homodimer. The same gene encodes both cytosolic and mitochondrial KARS enzymes (Shiba et al. 1997).
R-HSA-380234 (Reactome) HARS2 (mitochondrial histidyl tRNA synthetase) catalyzes the reaction of histidine, mitochondrial tRNA(His), and ATP to form His-tRNA(His), AMP, and pyrophosphate. The enzyme is a class II tRNA synthetase (O'Hanlon et al. 1995).
R-HSA-380239 (Reactome) SARS2 (mitochondrial seryl tRNA synthetase) catalyzes the reaction of serine, mitochondrial tRNA(Ser), and ATP to form Ser-tRNA(Ser), AMP, and pyrophosphate. SARS2 is a class II tRNA synthetase inferred from the biochemical properties of its bovine homologue to function as a dimer (Yokogawa et al. 2000).
R-HSA-380240 (Reactome) GARS (mitochondrial glycyl tRNA synthetase) catalyzes the reaction of glycine, tRNA(Gly), and ATP to form Gly-tRNA(Gly), AMP, and pyrophosphate. The enzyme, a class II tRNA synthetase, is a dimer. Cytosolic and mitochondrial glycyl tRNA synthetase enzymes are both encoded by the same gene; the cytosolic protein lacks an aminoterminal 54-residue sequence found in the mitochondrial protein. Mutations in GARS are associated with Charcot-Marie-Tooth disease (Antonellis et al. 2007).
R-HSA-380241 (Reactome) QARS (mitochondrial glutaminyl tRNA synthetase) catalyzes the reaction of glutamine, tRNA(Gln), and ATP to form Gln-tRNA(Gln), AMP, and pyrophosphate. The enzyme is a class I tRNA synthetase. The same gene encodes both the cytosolic and mitochondrial QARS enzymes (Lamour et al. 1994).
R-HSA-449937 (Reactome) Mitochondrial PPA2 (pyrophosphatase (inorganic) 2) catalyzes the hydrolysis of pyrophosphate to yield two molecules of orthophosphate. The enzyme requires Mg++ for activity (Curbo et al. 2006). The enzyme is inferred to be a homodimer by analogy to its cytosolic isoform.
R-HSA-71732 (Reactome) Cytosolic PPA1 (pyrophosphatase (inorganic) 1) catalyzes the hydrolysis of pyrophosphate to yield two molecules of orthophosphate. The enzyme, a homodimer, requires Mg++ for activity (Fisher et al. 1974; Pynes and Younathan 1967; Thuiller et al. 1978).
RARS2mim-catalysisR-HSA-380224 (Reactome)
SARS dimermim-catalysisR-HSA-379992 (Reactome)
SARS2 dimermim-catalysisR-HSA-380239 (Reactome)
Ser-tRNA(Ser)ArrowR-HSA-379992 (Reactome)
Ser-tRNA(Ser)ArrowR-HSA-380239 (Reactome)
SerR-HSA-379992 (Reactome)
TARS dimermim-catalysisR-HSA-380002 (Reactome)
TARS2(1-?)mim-catalysisR-HSA-380201 (Reactome)
Thr-tRNA(Thr)ArrowR-HSA-380002 (Reactome)
Thr-tRNA(Thr)ArrowR-HSA-380201 (Reactome)
Trp-tRNA(Trp)ArrowR-HSA-379977 (Reactome)
Trp-tRNA(Trp)ArrowR-HSA-380222 (Reactome)
Tyr-tRNA(Tyr)ArrowR-HSA-379980 (Reactome)
Tyr-tRNA(Tyr)ArrowR-HSA-380170 (Reactome)
VARS2mim-catalysisR-HSA-380199 (Reactome)
VARSmim-catalysisR-HSA-380042 (Reactome)
Val-tRNA(Val)ArrowR-HSA-380042 (Reactome)
Val-tRNA(Val)ArrowR-HSA-380199 (Reactome)
WARS dimermim-catalysisR-HSA-379977 (Reactome)
WARS2mim-catalysisR-HSA-380222 (Reactome)
YARS dimermim-catalysisR-HSA-379980 (Reactome)
YARS2 dimermim-catalysisR-HSA-380170 (Reactome)
aminoacyl-tRNA

synthetase

multienzyme complex		mim-catalysisR-HSA-379861 (Reactome)
aminoacyl-tRNA

synthetase

multienzyme complex		mim-catalysisR-HSA-379865 (Reactome)
aminoacyl-tRNA

synthetase

multienzyme complex		mim-catalysisR-HSA-379867 (Reactome)
aminoacyl-tRNA

synthetase

multienzyme complex		mim-catalysisR-HSA-379893 (Reactome)
aminoacyl-tRNA

synthetase

multienzyme complex		mim-catalysisR-HSA-379974 (Reactome)
aminoacyl-tRNA

synthetase

multienzyme complex		mim-catalysisR-HSA-379982 (Reactome)
aminoacyl-tRNA

synthetase

multienzyme complex		mim-catalysisR-HSA-379993 (Reactome)
aminoacyl-tRNA

synthetase

multienzyme complex		mim-catalysisR-HSA-379994 (Reactome)
aminoacyl-tRNA

synthetase

multienzyme complex		mim-catalysisR-HSA-380008 (Reactome)
tRNA(Ala)R-HSA-379864 (Reactome)
tRNA(Ala)R-HSA-380177 (Reactome)
tRNA(Arg)R-HSA-379993 (Reactome)
tRNA(Arg)R-HSA-380224 (Reactome)
tRNA(Asn)R-HSA-379996 (Reactome)
tRNA(Asn)R-HSA-380227 (Reactome)
tRNA(Asp)R-HSA-379867 (Reactome)
tRNA(Asp)R-HSA-380229 (Reactome)
tRNA(Cys)R-HSA-379887 (Reactome)
tRNA(Cys)R-HSA-380158 (Reactome)
tRNA(Gln)R-HSA-379982 (Reactome)
tRNA(Gln)R-HSA-380241 (Reactome)
tRNA(Glu)R-HSA-379861 (Reactome)
tRNA(Glu)R-HSA-380216 (Reactome)
tRNA(Gly)R-HSA-380048 (Reactome)
tRNA(Gly)R-HSA-380240 (Reactome)
tRNA(His)R-HSA-379844 (Reactome)
tRNA(His)R-HSA-380234 (Reactome)
tRNA(Ile)R-HSA-379893 (Reactome)
tRNA(Ile)R-HSA-380176 (Reactome)
tRNA(Leu)R-HSA-379974 (Reactome)
tRNA(Leu)R-HSA-380200 (Reactome)
tRNA(Lys)R-HSA-380008 (Reactome)
tRNA(Lys)R-HSA-380233 (Reactome)
tRNA(Met)R-HSA-379994 (Reactome)
tRNA(Met)R-HSA-380157 (Reactome)
tRNA(Phe)R-HSA-379848 (Reactome)
tRNA(Phe)R-HSA-380203 (Reactome)
tRNA(Pro)R-HSA-379865 (Reactome)
tRNA(Pro)R-HSA-380198 (Reactome)
tRNA(Ser)R-HSA-379992 (Reactome)
tRNA(Ser)R-HSA-380239 (Reactome)
tRNA(Thr)R-HSA-380002 (Reactome)
tRNA(Thr)R-HSA-380201 (Reactome)
tRNA(Trp)R-HSA-379977 (Reactome)
tRNA(Trp)R-HSA-380222 (Reactome)
tRNA(Tyr)R-HSA-379980 (Reactome)
tRNA(Tyr)R-HSA-380170 (Reactome)
tRNA(Val)R-HSA-380042 (Reactome)
tRNA(Val)R-HSA-380199 (Reactome)

Retrieved from "https://classic.wikipathways.org/index.php/Pathway:WP1938"
Personal tools