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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 <I>Aars</I> 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)., Authored: D'Eustachio, P, 2008-11-29 15:41:00, Edited: D'Eustachio, P, 2008-11-29 15:41:00, Reviewed: Antonellis, A, 2008-12-02 16:58:45
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alanine + tRNA(Ala) + ATP => Ala-tRNA(Ala) + AMP + pyrophosphate
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6.1.1.7
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