AARS (cytosolic alanyl tRNA synthetase) catalyzes the reaction of alanine, tRNA(ala), and ATP to form Ala tRNA(Ala), AMP, and pyrophosphate. The enzyme, a class II tRNA synthetase, is a monomer (Shiba et al. 1995). A mutation in the editing domain of the mouse Aars gene results in misincorporation of non-cognate amino acids into cellular proteins. This is associated with protein misfolding in Purkinje cells and a phenotype consistent with human ataxia (Lee et al. 2006).
Schimmel, P, Suzuki, N, Ripmaster, T, Nichols, R, Shiba, K, Noda, T, Plotz, P
Beebe, K, Schimmel, P, Sundberg, JP, Cook, SA, Nangle, LA, Davisson, MT, Ackerman, SL, Jang, J, Longo-Guess, CM, Lee, JW
alanine-tRNA ligase activity of AARS [cytosol]
© 2023 Reactome