Reactome: A Curated Pathway Database

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

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