Reactome: A Curated Pathway Database

aspartate + tRNA(Asp) + ATP => Asp-tRNA(Asp) + AMP + pyrophosphate (R-HSA-380229)

Species Homo sapiens


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).

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Compartment mitochondrial matrix
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Catalyst Activity
PhysicalEntity Activity Active Units
DARS2 dimer aspartate-tRNA ligase activity (0004815)