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Identification
HMDB Protein ID HMDBP10671
Secondary Accession Numbers
  • 16929
Name Alanine--tRNA ligase, mitochondrial
Synonyms
  1. AlaRS
  2. Alanine--tRNA ligase
  3. Alanyl-tRNA synthetase-like
  4. Alanyl-tRNA synthetase
Gene Name AARS2
Protein Type Enzyme
Biological Properties
General Function Involved in nucleotide binding
Specific Function Catalyzes the attachment of alanine to tRNA(Ala) in a two-step reaction: alanine is first activated by ATP to form Ala-AMP and then transferred to the acceptor end of tRNA(Ala). Also edits incorrectly charged tRNA(Ala) via its editing domain (By similarity).
Pathways
  • Alanine Metabolism
  • Aminoacyl-tRNA biosynthesis
  • Lactic Acidemia
  • Primary Hyperoxaluria Type I
  • Pyruvate Carboxylase Deficiency
Reactions
Adenosine triphosphate + L-Alanine + tRNA(Ala) → Adenosine monophosphate + Pyrophosphate + L-alanyl-tRNA(Ala) details
Adenosine triphosphate + L-Alanine + tRNA(Ala) → Adenosine monophosphate + Pyrophosphate + L-Alanyl-tRNA details
GO Classification
Biological Process
mitochondrial alanyl-tRNA aminoacylation
mitochondrial respiratory chain complex assembly
Cellular Component
mitochondrion
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
alanine-trna ligase activity
nucleic acid binding
ligase activity, forming carbon-oxygen bonds
ligase activity, forming aminoacyl-trna and related compounds
aminoacyl-trna ligase activity
Molecular Function
metal ion binding
ATP binding
alanine-tRNA ligase activity
tRNA binding
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
alanyl-trna aminoacylation
biosynthetic process
Cellular Location
  1. Mitochondrion matrix
Gene Properties
Chromosome Location 6
Locus 6p21.1
SNPs AARS2
Gene Sequence
>2958 bp
ATGGCAGCGTCAGTGGCAGCTGCAGCCCGGAGGCTGCGGCGGGCCATTCGAAGGTCGCCC
GCATGGCGGGGCCTCAGCCATCGGCCGCTCTCATCGGAGCCCCCTGCAGCCAAGGCCTCG
GCCGTGAGGGCCGCCTTTCTGAACTTCTTTCGGGACCGCCATGGCCACCGGCTGGTGCCC
TCCGCTTCCGTGCGGCCCCGCGGCGACCCCAGTTTGCTTTTTGTCAATGCGGGCATGAAC
CAGTTCAAGCCAATCTTTCTGGGCACCGTGGATCCACGAAGCGAGATGGCAGGCTTCCGA
CGTGTGGCCAACAGCCAGAAATGTGTGAGAGCTGGAGGACACCATAACGACCTGGAAGAT
GTGGGTCGAGACCTTTCCCATCATACCTTCTTTGAAATGCTTGGCAATTGGGCCTTTGGG
GGTGAATATTTTAAGGAGGAGGCTTGTAACATGGCCTGGGAACTGCTGACTCAGGTCTAT
GGGATCCCTGAGGAAAGGCTCTGGATCTCCTACTTTGATGGTGACCCCAAGGCAGGGCTG
GACCCAGACCTGGAGACCAGGGACATCTGGCTGAGCTTAGGGGTGCCTGCTAGCCGTGTG
CTTTCCTTTGGACCACAAGAGAACTTCTGGGAGATGGGGGATACTGGCCCTTGTGGGCCC
TGTACTGAGATCCACTACGACCTTGCTGGTGGGGTGGGAGCCCCCCAGCTGGTAGAGCTT
TGGAACCTGGTCTTCATGCAACACAACAGAGAGGCAGATGGAAGCCTGCAGCCCCTGCCC
CAGCGGCATGTGGACACAGGAATGGGCCTGGAAAGGCTGGTGGCTGTGCTGCAAGGCAAA
CACTCCACCTATGACACTGACCTCTTTTCCCCGCTGCTCAACGCCATACAGCAGGGCTGC
AGGGCACCCCCTTACTTGGGCCGAGTAGGGGTGGCAGACGAGGGGCGCACAGACACAGCG
TACCGCGTGGTGGCTGACCACATCCGCACACTCAGTGTCTGCATCTCTGATGGCGTCTTC
CCTGGGATGTCAGGTCCCCCGCTGGTTCTTCGTCGGATCCTGCGTCGAGCTGTGCGTTTC
TCCATGGAGATCTTAAAGGCACCACCTGGCTTCCTAGGCAGCCTGGTACCTGTAGTGGTG
GAGACACTGGGAGATGCTTATCCAGAACTGCAAAGGAACTCAGCCCAGATCGCCAACCTG
GTGTCAGAGGACGAGGCAGCCTTCCTGGCCTCCCTGGAGCGGGGTAGGCGGATCATTGAT
CGGACTCTGAGGACCCTGGGGCCTTCAGATATGTTCCCTGCTGAAGTGGCCTGGTCCTTG
TCACTGTGTGGAGACCTGGGACTCCCCTTGGACATGGTAGAGCTGATGCTGGAGGAGAAA
GGGGTCCAGCTAGACTCCGCTGGACTGGAGCGGTTGGCCCAAGAGGAGGCCCAGCACCGG
GCACGGCAGGCTGAGCCAGTTCAGAAGCAGGGATTGTGGCTTGATGTCCATGCGCTTGGG
GAGCTGCAGCGCCAAGGAGTGCCCCCAACTGACGACAGCCCCAAGTACAACTACTCCCTG
CGACCCAGCGGAAGTTATGAGTTCGGCACCTGTGAGGCCCAGGTGTTGCAACTGTATACA
GAGGACGGGACAGCAGTGGCCTCCGTGGGGAAAGGCCAGCGCTGTGGCCTCCTCTTGGAC
AGGACCAACTTCTACGCAGAACAGGGGGGCCAGGCTTCAGACCGTGGCTACCTGGTGCGG
GCAGGGCAAGAGGACGTGCTGTTCCCAGTAGCCCGGGCCCAGGTCTGTGGAGGTTTCATC
CTGCATGAGGCAGTAGCCCCTGAGTGCCTGCGGTTAGGGGACCAGGTGCAGCTGCATGTG
GATGAGGCCTGGCGTCTAGGCTGCATGGCGAAGCATACGGCCACCCACCTGCTGAACTGG
GCACTGAGGCAGACCCTGGGCCCTGGCACAGAGCAGCAGGGCTCCCATCTCAATCCTGAG
CAGCTGCGCTTGGATGTGACCACCCAGACCCCATTGACCCCAGAGCAGCTCCGGGCAGTG
GAGAACACTGTGCAGGAGGCCGTGGGGCAGGATGAGGCTGTGTACATGGAGGAGGTGCCC
CTGGCGCTCACTGCCCAGGTCCCTGGCCTGCGCTCTCTGGATGAGGTTTACCCAGACCCT
GTGCGGGTGGTATCAGTGGGGGTGCCCGTGGCCCATGCATTGGACCCAGCCTCCCAAGCC
GCACTGCAGACCTCTGTGGAGCTATGCTGTGGGACGCACCTGTTACGTACTGGGGCTGTA
GGGGACCTGGTTATCATCGGGGACCGCCAGCTTTCCAAGGGCACTACCCGCCTGCTGGCC
GTCACTGGGGAGCAGGCCCAGCAGGCCCGAGAGCTAGGCCAGAGCCTGGCCCAGGAAGTG
AAAGCGGCCACTGAGCGGCTGAGTCTGGGGAGCCGGGATGTGGCGGAGGCACTGAGGCTG
TCCAAGGACATAGGACGACTCATTGAAGCTGTGGAAACTGCTGTGATGCCCCAGTGGCAG
CGGCGGGAGCTGCTGGCCACAGTGAAGATGCTGCAGCGGCGTGCCAACACTGCCATCCGT
AAGCTGCAAATGGGACAGGCTGCAAAGAAAACTCAGGAGCTGCTGGAGCGGCACTCGAAG
GGGCCTCTGATTGTGGACACAGTCTCTGCTGAGTCTCTCTCAGTGCTGGTGAAGGTGGTA
CGGCAGCTGTGTGAGCAGGCCCCCAGCACGTCTGTGCTCCTACTCAGCCCCCAGCCCATG
GGGAAGGTGCTGTGTGCCTGTCAGGTGGCCCAGGGTGCCATGCCCACCTTCACAGCAGAG
GCCTGGGCACTGGCAGTGTGCAGCCACATGGGGGGCAAGGCGTGGGGCTCGCGAGTGGTG
GCCCAAGGCACCGGAAGCACTACTGACCTGGAAGCTGCCCTCAGTATAGCCCAAACCTAT
GCCCTCAGCCAGCTCTGA
Protein Properties
Number of Residues 985
Molecular Weight 107325.46
Theoretical pI 6.261
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Probable alanyl-tRNA synthetase, mitochondrial
MAASVAAAARRLRRAIRRSPAWRGLSHRPLSSEPPAAKASAVRAAFLNFFRDRHGHRLVP
SASVRPRGDPSLLFVNAGMNQFKPIFLGTVDPRSEMAGFRRVANSQKCVRAGGHHNDLED
VGRDLSHHTFFEMLGNWAFGGEYFKEEACNMAWELLTQVYGIPEERLWISYFDGDPKAGL
DPDLETRDIWLSLGVPASRVLSFGPQENFWEMGDTGPCGPCTEIHYDLAGGVGAPQLVEL
WNLVFMQHNREADGSLQPLPQRHVDTGMGLERLVAVLQGKHSTYDTDLFSPLLNAIQQGC
RAPPYLGRVGVADEGRTDTAYRVVADHIRTLSVCISDGIFPGMSGPPLVLRRILRRAVRF
SMEILKAPPGFLGSLVPVVVETLGDAYPELQRNSAQIANLVSEDEAAFLASLERGRRIID
RTLRTLGPSDMFPAEVAWSLSLCGDLGLPLDMVELMLEEKGVQLDSAGLERLAQEEAQHR
ARQAEPVQKQGLWLDVHALGELQRQGVPPTDDSPKYNYSLRPSGSYEFGTCEAQVLQLYT
EDGTAVASVGKGQRCGLLLDRTNFYAEQGGQASDRGYLVRAGQEDVLFPVARAQVCGGFI
LHEAVAPECLRLGDQVQLHVDEAWRLGCMAKHTATHLLNWALRQTLGPGTEQQGSHLNPE
QLRLDVTTQTPLTPEQLRAVENTVQEAVGQDEAVYMEEVPLALTAQVPGLRSLDEVYPDP
VRVVSVGVPVAHALDPASQAALQTSVELCCGTHLLRTGAVGDLVIIGDRQLSKGTTRLLA
VTGEQAQQARELGQSLAQEVKAATERLSLGSRDVAEALRLSKDIGRLIEAVETAVMPQWQ
RRELLATVKMLQRRANTAIRKLQMGQAAKKTQELLERHSKGPLIVDTVSAESLSVLVKVV
RQLCEQAPSTSVLLLSPQPMGKVLCACQVAQGAMPTFTAEAWALAVCSHMGGKAWGSRVV
AQGTGSTTDLEAALSIAQTYALSQL
GenBank ID Protein 38569417
UniProtKB/Swiss-Prot ID Q5JTZ9
UniProtKB/Swiss-Prot Entry Name SYAM_HUMAN
PDB IDs Not Available
GenBank Gene ID NM_020745.2
GeneCard ID AARS2
GenAtlas ID AARS2
HGNC ID HGNC:21022
References
General References
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  3. 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 ]
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