ID:SYAM_HUMAN DESCRIPTION: RecName: Full=Alanine--tRNA ligase, mitochondrial; EC=6.1.1.7; AltName: Full=Alanyl-tRNA synthetase; Short=AlaRS; Flags: Precursor; 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). CATALYTIC ACTIVITY: ATP + L-alanine + tRNA(Ala) = AMP + diphosphate + L-alanyl-tRNA(Ala). COFACTOR: Binds 1 zinc ion per subunit (Potential). SUBUNIT: Monomer (By similarity). SUBCELLULAR LOCATION: Mitochondrion. DOMAIN: Consists of three domains; the N-terminal catalytic domain, the editing domain and the C-terminal C-Ala domain. The editing domain removes incorrectly charged amino acids, while the C-Ala domain, along with tRNA(Ala), serves as a bridge to cooperatively bring together the editing and aminoacylation centers thus stimulating deacylation of misacylated tRNAs (By similarity). DISEASE: Defects in AARS2 are the cause of combined oxidative phosphorylation deficiency type 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. SIMILARITY: Belongs to the class-II aminoacyl-tRNA synthetase family. SEQUENCE CAUTION: Sequence=BAA86584.1; Type=Erroneous initiation;
The RNAfold program from the Vienna RNA Package is used to perform the secondary structure predictions and folding calculations. The estimated folding energy is in kcal/mol. The more negative the energy, the more secondary structure the RNA is likely to have.
Pfam Domains: PF01411 - tRNA synthetases class II (A) PF07973 - Threonyl and Alanyl tRNA synthetase second additional domain
SCOP Domains: 101353 - Putative anticodon-binding domain of alanyl-tRNA synthetase (AlaRS) 50447 - Translation proteins 55681 - Class II aaRS and biotin synthetases 55186 - ThrRS/AlaRS common domain
ModBase Predicted Comparative 3D Structure on Q5JTZ9
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Orthologous Genes in Other Species
Orthologies between human, mouse, and rat are computed by taking the best BLASTP hit, and filtering out non-syntenic hits. For more distant species reciprocal-best BLASTP hits are used. Note that the absence of an ortholog in the table below may reflect incomplete annotations in the other species rather than a true absence of the orthologous gene.
US Server
Sequence and Links to Tools and Databases 
Common Gene Haplotype Alleles 
