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).
|DARS2 dimer||aspartate-tRNA ligase activity (0004815)|
|15779907||Toward the full set of human mitochondrial aminoacyl-tRNA synthetases: characterization of AspRS and TyrRS||Biochemistry||2005|
|17384640||Mitochondrial aspartyl-tRNA synthetase deficiency causes leukoencephalopathy with brain stem and spinal cord involvement and lactate elevation||Nat Genet||2007|