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Identification
HMDB Protein ID HMDBP07360
Secondary Accession Numbers
  • 12994
Name Probable arginine--tRNA ligase, mitochondrial
Synonyms
  1. ArgRS
  2. Arginine--tRNA ligase
  3. Arginyl-tRNA synthetase-like
  4. Arginyl-tRNA synthetase
Gene Name RARS2
Protein Type Enzyme
Biological Properties
General Function Involved in nucleotide binding
Specific Function Not Available
Pathways
  • Aminoacyl-tRNA biosynthesis
  • Arginine and proline metabolism
  • Arginine: Glycine Amidinotransferase Deficiency (AGAT Deficiency)
  • Creatine deficiency, guanidinoacetate methyltransferase deficiency
  • Guanidinoacetate Methyltransferase Deficiency (GAMT Deficiency)
  • Hyperornithinemia with gyrate atrophy (HOGA)
  • Hyperornithinemia-hyperammonemia-homocitrullinuria [HHH-syndrome]
  • Hyperprolinemia Type I
  • Hyperprolinemia Type II
  • L-arginine:glycine amidinotransferase deficiency
  • Ornithine Aminotransferase Deficiency (OAT Deficiency)
  • Prolidase Deficiency (PD)
  • Prolinemia Type II
Reactions
Adenosine triphosphate + L-Arginine + tRNA(Arg) → Adenosine monophosphate + Pyrophosphate + L-arginyl-tRNA(Arg) details
Adenosine triphosphate + L-Arginine + tRNA(Arg) → Adenosine monophosphate + Pyrophosphate + L-Arginyl-tRNA(Arg) details
GO Classification
Biological Process
tRNA aminoacylation for protein translation
arginyl-tRNA aminoacylation
Cellular Component
mitochondrial matrix
Component
cell part
intracellular part
cytoplasm
Function
binding
nucleotide binding
catalytic activity
nucleoside binding
purine nucleoside binding
adenyl nucleotide binding
adenyl ribonucleotide binding
atp binding
ligase activity
arginine-trna ligase activity
ligase activity, forming carbon-oxygen bonds
ligase activity, forming aminoacyl-trna and related compounds
aminoacyl-trna ligase activity
Molecular Function
ATP binding
arginine-tRNA ligase activity
Process
rna metabolic process
ncrna metabolic process
trna metabolic process
trna aminoacylation
trna aminoacylation for protein translation
macromolecule biosynthetic process
cellular macromolecule biosynthetic process
translation
metabolic process
macromolecule metabolic process
cellular macromolecule metabolic process
arginyl-trna aminoacylation
biosynthetic process
Cellular Location
  1. Mitochondrion matrix
Gene Properties
Chromosome Location 6
Locus 6q16.1
SNPs RARS2
Gene Sequence
>1737 bp
ATGGCGTGCGGCTTTCGCCGCGCTATTGCTTGCCAGCTTTCCAGAGTGTTGAATCTTCCA
CCAGAAAACTTGATCACATCAATATCTGCAGTTCCAATTTCCCAAAAAGAAGAAGTAGCT
GATTTTCAGCTTTCTGTGGATCCTTTATTGGAAAAAGACAATGACCATTCAAGACCAGAT
ATTCAAGTTCAAGCCAAGAGACTAGCAGAGAAGCTAAGATGTGATACAGTGGTGAGTGAA
ATCAGTACTGGTCAAAGGACTGTAAATTTCAAAATAAACAGAGAGCTCTTAACAAAGACA
GTGCTACAACAAGTAATTGAAGATGGCTCAAAATATGGATTAAAAAGTGAACTTTTCTCT
GGACTTCCCCAGAAGAAGATTGTGGTTGAATTCAGTTCACCTAATGTTGCCAAAAAATTT
CATGTTGGACATTTGCGTTCTACCGTCATAGGAAATTTTATAGCAAATCTCAAAGAAGCT
TTAGGACATCAAGTAATAAGAATAAATTACCTTGGCGATTGGGGCATGCAGTTTGGTCTT
CTGGGAACTGGCTTCCAGCTGTTTGGCTATGAGGAAAAACTGCAGTCCAATCCTCTACAG
CATCTCTTTGAAGTTTATGTACAAGTTAATAAAGAAGCAGCGGATGATAAAAGTGTAGCA
AAAGCAGCACAGGAGTTCTTCCAACGATTGGAACTGGGCGATGTGCAAGCACTTTCACTG
TGGCAAAAATTTCGGGACTTGAGCATTGAAGAGTACATTCGGGTTTACAAGCGTCTGGGA
GTATATTTTGATGAATATTCAGGAGAATCATTTTATCGTGAAAAATCTCAAGAGGTCTTA
AAGTTGCTGGAGAGTAAAGGACTCCTACTGAAAACAATAAAAGGAACGGCTGTAGTAGAT
CTCTCTGGGAATGGCGACCCCTCCTCAATTTGTACTGTAATGCGAAGTGATGGGACTTCT
CTCTATGCAACCAGAGATCTTGCAGCTGCTGTAGATCGAATGGACAAGTATAATTTTGAT
ACAATGATATATGTGACAGATAAAGGACAAAAAAAGCATTTTCAGCAAGTATTCCAAATG
CTGAAGATCATGGGATATGACTGGGCAGAAAGGTGCCAGCACGTGCCCTTTGGAGTAGTA
CAGGGAATGAAGACTCGAAGAGGAGATGTCACTTTCCTGGAAGATGTTTTAAATGAGATT
CAATTAAGGATGCTACAGAACATGGCTTCAATTAAGACAACTAAAGAACTCAAGAACCCA
CAAGAGACTGCAGAGAGGGTCGGGCTCGCAGCACTCATTATTCAGGACTTCAAAGGTTTA
CTCTTATCTGACTACAAGTTCAGCTGGGATCGTGTTTTCCAGAGTCGCGGGGACACAGGA
GTCTTCCTACAGTACACACACGCCCGCCTCCACAGTTTGGAAGAGACTTTTGGATGTGGG
TACCTGAATGACTTCAACACTGCTTGTTTACAAGAGCCACAGTCTGTTTCAATTCTTCAG
CATCTTCTCAGGTTCGACGAGGTGCTTTATAAATCATCTCAGGACTTTCAACCCAGGCAT
ATCGTCAGTTACCTTCTAACTTTAAGTCATCTTGCAGCTGTGGCACACAAAACACTACAA
ATAAAAGATAGTCCTCCTGAAGTGGCTGGGGCCAGACTTCATCTTTTCAAAGCTGTCCGT
TCTGTCCTAGCCAATGGAATGAAACTTCTTGGAATAACACCTGTATGTAGGATGTAA
Protein Properties
Number of Residues 578
Molecular Weight 65504.855
Theoretical pI 8.212
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Probable arginyl-tRNA synthetase, mitochondrial
MACGFRRAIACQLSRVLNLPPENLITSISAVPISQKEEVADFQLSVDSLLEKDNDHSRPD
IQVQAKRLAEKLRCDTVVSEISTGQRTVNFKINRELLTKTVLQQVIEDGSKYGLKSELFS
GLPQKKIVVEFSSPNVAKKFHVGHLRSTIIGNFIANLKEALGHQVIRINYLGDWGMQFGL
LGTGFQLFGYEEKLQSNPLQHLFEVYVQVNKEAADDKSVAKAAQEFFQRLELGDVQALSL
WQKFRDLSIEEYIRVYKRLGVYFDEYSGESFYREKSQEVLKLLESKGLLLKTIKGTAVVD
LSGNGDPSSICTVMRSDGTSLYATRDLAAAIDRMDKYNFDTMIYVTDKGQKKHFQQVFQM
LKIMGYDWAERCQHVPFGVVQGMKTRRGDVTFLEDVLNEIQLRMLQNMASIKTTKELKNP
QETAERVGLAALIIQDFKGLLLSDYKFSWDRVFQSRGDTGVFLQYTHARLHSLEETFGCG
YLNDFNTACLQEPQSVSILQHLLRFDEVLYKSSQDFQPRHIVSYLLTLSHLAAVAHKTLQ
IKDSPPEVAGARLHLFKAVRSVLANGMKLLGITPVCRM
GenBank ID Protein 10435519
UniProtKB/Swiss-Prot ID Q5T160
UniProtKB/Swiss-Prot Entry Name SYRM_HUMAN
PDB IDs Not Available
GenBank Gene ID AK023550
GeneCard ID RARS2
GenAtlas ID RARS2
HGNC ID HGNC:21406
References
General References
  1. Mungall AJ, Palmer SA, Sims SK, Edwards CA, Ashurst JL, Wilming L, Jones MC, Horton R, Hunt SE, Scott CE, Gilbert JG, Clamp ME, Bethel G, Milne S, Ainscough R, Almeida JP, Ambrose KD, Andrews TD, Ashwell RI, Babbage AK, Bagguley CL, Bailey J, Banerjee R, Barker DJ, Barlow KF, Bates K, Beare DM, Beasley H, Beasley O, Bird CP, Blakey S, Bray-Allen S, Brook J, Brown AJ, Brown JY, Burford DC, Burrill W, Burton J, Carder C, Carter NP, Chapman JC, Clark SY, Clark G, Clee CM, Clegg S, Cobley V, Collier RE, Collins JE, Colman LK, Corby NR, Coville GJ, Culley KM, Dhami P, Davies J, Dunn M, Earthrowl ME, Ellington AE, Evans KA, Faulkner L, Francis MD, Frankish A, Frankland J, French L, Garner P, Garnett J, Ghori MJ, Gilby LM, Gillson CJ, Glithero RJ, Grafham DV, Grant M, Gribble S, Griffiths C, Griffiths M, Hall R, Halls KS, Hammond S, Harley JL, Hart EA, Heath PD, Heathcott R, Holmes SJ, Howden PJ, Howe KL, Howell GR, Huckle E, Humphray SJ, Humphries MD, Hunt AR, Johnson CM, Joy AA, Kay M, Keenan SJ, Kimberley AM, King A, Laird GK, Langford C, Lawlor S, Leongamornlert DA, Leversha M, Lloyd CR, Lloyd DM, Loveland JE, Lovell J, Martin S, Mashreghi-Mohammadi M, Maslen GL, Matthews L, McCann OT, McLaren SJ, McLay K, McMurray A, Moore MJ, Mullikin JC, Niblett D, Nickerson T, Novik KL, Oliver K, Overton-Larty EK, Parker A, Patel R, Pearce AV, Peck AI, Phillimore B, Phillips S, Plumb RW, Porter KM, Ramsey Y, Ranby SA, Rice CM, Ross MT, Searle SM, Sehra HK, Sheridan E, Skuce CD, Smith S, Smith M, Spraggon L, Squares SL, Steward CA, Sycamore N, Tamlyn-Hall G, Tester J, Theaker AJ, Thomas DW, Thorpe A, Tracey A, Tromans A, Tubby B, Wall M, Wallis JM, West AP, White SS, Whitehead SL, Whittaker H, Wild A, Willey DJ, Wilmer TE, Wood JM, Wray PW, Wyatt JC, Young L, Younger RM, Bentley DR, Coulson A, Durbin R, Hubbard T, Sulston JE, Dunham I, Rogers J, Beck S: The DNA sequence and analysis of human chromosome 6. Nature. 2003 Oct 23;425(6960):805-11. [PubMed:14574404 ]
  2. Ota T, Suzuki Y, Nishikawa T, Otsuki T, Sugiyama T, Irie R, Wakamatsu A, Hayashi K, Sato H, Nagai K, Kimura K, Makita H, Sekine M, Obayashi M, Nishi T, Shibahara T, Tanaka T, Ishii S, Yamamoto J, Saito K, Kawai Y, Isono Y, Nakamura Y, Nagahari K, Murakami K, Yasuda T, Iwayanagi T, Wagatsuma M, Shiratori A, Sudo H, Hosoiri T, Kaku Y, Kodaira H, Kondo H, Sugawara M, Takahashi M, Kanda K, Yokoi T, Furuya T, Kikkawa E, Omura Y, Abe K, Kamihara K, Katsuta N, Sato K, Tanikawa M, Yamazaki M, Ninomiya K, Ishibashi T, Yamashita H, Murakawa K, Fujimori K, Tanai H, Kimata M, Watanabe M, Hiraoka S, Chiba Y, Ishida S, Ono Y, Takiguchi S, Watanabe S, Yosida M, Hotuta T, Kusano J, Kanehori K, Takahashi-Fujii A, Hara H, Tanase TO, Nomura Y, Togiya S, Komai F, Hara R, Takeuchi K, Arita M, Imose N, Musashino K, Yuuki H, Oshima A, Sasaki N, Aotsuka S, Yoshikawa Y, Matsunawa H, Ichihara T, Shiohata N, Sano S, Moriya S, Momiyama H, Satoh N, Takami S, Terashima Y, Suzuki O, Nakagawa S, Senoh A, Mizoguchi H, Goto Y, Shimizu F, Wakebe H, Hishigaki H, Watanabe T, Sugiyama A, Takemoto M, Kawakami B, Yamazaki M, Watanabe K, Kumagai A, Itakura S, Fukuzumi Y, Fujimori Y, Komiyama M, Tashiro H, Tanigami A, Fujiwara T, Ono T, Yamada K, Fujii Y, Ozaki K, Hirao M, Ohmori Y, Kawabata A, Hikiji T, Kobatake N, Inagaki H, Ikema Y, Okamoto S, Okitani R, Kawakami T, Noguchi S, Itoh T, Shigeta K, Senba T, Matsumura K, Nakajima Y, Mizuno T, Morinaga M, Sasaki M, Togashi T, Oyama M, Hata H, Watanabe M, Komatsu T, Mizushima-Sugano J, Satoh T, Shirai Y, Takahashi Y, Nakagawa K, Okumura K, Nagase T, Nomura N, Kikuchi H, Masuho Y, Yamashita R, Nakai K, Yada T, Nakamura Y, Ohara O, Isogai T, Sugano S: Complete sequencing and characterization of 21,243 full-length human cDNAs. Nat Genet. 2004 Jan;36(1):40-5. Epub 2003 Dec 21. [PubMed:14702039 ]
  3. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [PubMed:15489334 ]
  4. Bonnefond L, Fender A, Rudinger-Thirion J, Giege R, Florentz C, Sissler M: Toward the full set of human mitochondrial aminoacyl-tRNA synthetases: characterization of AspRS and TyrRS. Biochemistry. 2005 Mar 29;44(12):4805-16. [PubMed:15779907 ]
  5. 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. Am J Hum Genet. 2007 Oct;81(4):857-62. Epub 2007 Aug 24. [PubMed:17847012 ]