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

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

Stable Identifier

R-HSA-380229

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

ReviewStatus

5/5

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

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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