Glutamyl tRNA Synthetase 2, Mitochondrial (EARS2)

By sequencing clones obtained from a size-fractionated fetal brain cDNA library, Nagase et al. (2001) cloned EARS2. The transcript has repetitive elements in its 3-prime end, and the deduced protein contains 519 amino acids. RT-PCR ELISA detected robust EARS2 expression in all adult and fetal tissues and specific adult brain regions examined.By searching databases for aminoacyl-tRNA synthetases containing a mitochondrial targeting sequence, Bonnefond et al. (2005) identified EARS2, which the called mitochondrial GLURS. The deduced 523-amino acid protein has an N-terminal mitochondrial targeting signal with a predicted cleavage site after residue 25. EARS2 has characteristics of a class I aminoacyl-tRNA synthetase, including a classical Rossmann fold.