background:
The protein encoded by this gene belongs to the class-II aminoacyl-tRNA synthetase family. It is a mitochondrial enzyme that specifically aminoacylates aspartyl-tRNA. Mutations in this gene are associated with leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation (LBSL). [provided by RefSeq, Nov 2009]
Function:
Aspartyl-tRNA synthetase (DARS) charges its cognate tRNA with aspartate during protein biosynthesis. Mammalian synthetases differ from those of prokaryotes and lower eukaryotes in that they associate as multienzyme complexes. Defects in DARS2 are a cause of leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation.
Subcellular Location:
Mitochondrion matrix.
Similarity:
Belongs to the class-II aminoacyl-tRNA synthetase family.
Database links:
Entrez Gene: 55157 Human
Entrez Gene: 538772 Cow
Entrez Gene: 226539 Mouse
Entrez Gene: 304919 Rat
GenBank: NM_018122.3 Human
Omim: 610956 Human
SwissProt: A6QPU5 Cow
SwissProt: Q6PI48 Human
SwissProt: Q8BIP0 Mouse
SwissProt: Q3KRD0 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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