Reaction: aspartate + tRNA(Asp) + ATP => Asp-tRNA(Asp) + AMP + pyrophosphate
- in pathway: Mitochondrial tRNA aminoacylation
DARS2 (mitochondrial aspartyl tRNA synthetase) catalyzes the reaction of aspartate, mitochondrial tRNA(Asp), and ATP to form Asp tRNA(Asp), AMP, and pyrophosphate. The enzyme, a class II tRNA synthetase, is a homodimer (Bonnefond et al. 2005). Homozygosity for DARS2 mutations is associated with leukoencephalopathy with brainstem and spinal cord involvement plus lactate elevation (Scheper et al 2007).
Reaction - small molecule participants:
AMP [mitochondrial matrix]
PPi [mitochondrial matrix]
ATP [mitochondrial matrix]
L-Asp [mitochondrial matrix]
Reactome.org reaction link: R-HSA-380229
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Reaction input - small molecules:
ATP(4-)
L-aspartate(1-)
Reaction output - small molecules:
adenosine 5'-monophosphate(2-)
diphosphate(3-)
Reactome.org link: R-HSA-380229