Reactome: A Curated Pathway Database
Attention
The EBI data centre will be shutting down from the afternoon (BST) of Friday 26th August until the afternoon of Tuesday 30th August 2016 for essential maintenance. This might have an impact on some Reactome services and we apologize for any inconvenience.

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

Stable Identifier
R-HSA-380229
Type
Reaction
Species
Homo sapiens
Compartment
Locations in the PathwayBrowser
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).

Literature References
Participants
Participant Of
This entity is regulated by:
Title Physical Entity Activity
aspartate-tRNA ligase activity of DARS2 dimer [mitochondrial matrix] DARS2 dimer [mitochondrial matrix] aspartate-tRNA ligase activity (0004815)
Orthologous Events