Homo sapiens Protein: AARS2
Summary
InnateDB Protein IDBP-89595.6
Last Modified 2014-10-13 [Report errors or provide feedback]
Gene Symbol AARS2
Protein Name alanyl-tRNA synthetase 2, mitochondrial (putative)
Synonyms
Species Homo sapiens
Ensembl Protein ENSP00000244571
InnateDB Gene IDBG-89593 (AARS2)
Protein Structure
UniProt Annotation
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. {ECO:0000255HAMAP-Rule:MF_03133}.
Subcellular Localization Mitochondrion {ECO:0000255HAMAP- Rule:MF_03133, ECO:0000269PubMed:21549344}.
Disease Associations Combined oxidative phosphorylation deficiency 8 (COXPD8) [MIM:614096]: A mitochondrial disease characterized by a lethal infantile hypertrophic cardiomyopathy, generalized muscle dysfunction and some neurologic involvement. The liver is not affected. {ECO:0000269PubMed:21549344}. Note=The disease is caused by mutations affecting the gene represented in this entry.
Tissue Specificity
Comments
Interactions
Number of Interactions This gene and/or its encoded proteins are associated with 6 experimentally validated interaction(s) in this database.
Experimentally validated
Total 6 [view]
Protein-Protein 6 [view]
Protein-DNA 0
Protein-RNA 0
DNA-DNA 0
RNA-RNA 0
DNA-RNA 0
Gene Ontology

Molecular Function
Accession GO Term
GO:0000049 tRNA binding
GO:0000166 nucleotide binding
GO:0003676 nucleic acid binding
GO:0004813 alanine-tRNA ligase activity
GO:0005524 ATP binding
GO:0008270 zinc ion binding
GO:0016876 ligase activity, forming aminoacyl-tRNA and related compounds
Biological Process
GO:0006418 tRNA aminoacylation for protein translation
GO:0006419 alanyl-tRNA aminoacylation
GO:0010467 gene expression
GO:0033108 mitochondrial respiratory chain complex assembly
GO:0043039 tRNA aminoacylation
GO:0070143 mitochondrial alanyl-tRNA aminoacylation
Cellular Component
GO:0005737 cytoplasm
GO:0005739 mitochondrion
Protein Structure and Domains
PDB ID
InterPro IPR002318 Alanine-tRNA ligase, class IIc
IPR012947 Threonyl/alanyl tRNA synthetase, SAD
IPR018162 Alanine-tRNA ligase, class IIc, anti-codon-binding domain
IPR018163 Threonyl/alanyl tRNA synthetase, class II-like, putative editing domain
IPR018164 Alanyl-tRNA synthetase, class IIc, N-terminal
IPR018165 Alanyl-tRNA synthetase, class IIc, core domain
PFAM PF07973
PF01411
PRINTS PR00980
PIRSF
SMART SM00863
TIGRFAMs
Post-translational Modifications
Modification
Cross-References
SwissProt Q5JTZ9
PhosphoSite PhosphoSite-Q5JTZ9
TrEMBL
UniProt Splice Variant
Entrez Gene 57505
UniGene Hs.158381
RefSeq NP_065796
HUGO HGNC:21022
OMIM 612035
CCDS CCDS34464
HPRD 12397
IMGT
EMBL AB033096 AL353588 BC013593 BC033169 BC131728
GenPept AAH13593 AAH33169 AAI31729 BAA86584 CAI40747