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

Stable Identifier
R-HSA-380229
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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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
PubMed ID Title Journal Year
17384640 Mitochondrial aspartyl-tRNA synthetase deficiency causes leukoencephalopathy with brain stem and spinal cord involvement and lactate elevation

Smet, J, van der Klok, T, Krageloh-Mann, I, Sissler, M, Florentz, C, Pronk, JC, Smeitink, JA, Muravina, TI, Van Coster, R, Scheper, GC, van Andel, RJ, van der Knaap, MS, Schiffmann, R, van Berkel, CG, Serkov, SV, Bugiani, M, Uziel, G

Nat Genet 2007
15779907 Toward the full set of human mitochondrial aminoacyl-tRNA synthetases: characterization of AspRS and TyrRS

Florentz, C, Giege, R, Fender, A, Bonnefond, L, Sissler, M, Rudinger-Thirion, J

Biochemistry 2005
Participants
Participates
Catalyst Activity

aspartate-tRNA ligase activity of DARS2 dimer [mitochondrial matrix]

Orthologous Events
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