Aspartate-tRNA ligase, mitochondrial; mitochondrial Aspartyl-tRNA synthetase; AspRS2 (DARS2)

Function

-..........-

ATP + L-aspartate + tRNA(Asp)
        <-->
AMP + diphosphate + L-aspartyl-tRNA(Asp)

Structure

• belongs to the class-II aminoacyl-tRNA synthetase family

Compartment

mitochondrial matrix

Pathology

• defects in DARS2 are a cause of leukoencephalopathy with brainstem & spinal cord involvement & lactate elevation (LBSL)

More general terms

• amino acyl tRNA synthetase-2
• mitochondrial protein

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=55157
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:55157
• OMIM: https://mirror.omim.org/entry/610956
• OMIM: https://mirror.omim.org/entry/611105
• UniProt: http://www.uniprot.org/uniprot/Q6PI48.html