Reactome: A Curated Pathway Database

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

Species Homo sapiens

Summation

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).

Locations in the PathwayBrowser
Additional Information
Compartment mitochondrial matrix
Components of this entry
Input entries
Output entries
Catalyst Activity
PhysicalEntity Activity Active Units
DARS2 dimer aspartate-tRNA ligase activity (0004815)