2012). In addition, a mutation in an editing‐defective form of alanyl tRNA synthetase causes ataxia and neurodegeneration in mouse (Lee et al. 2006), and mutations in the gene encoding glutamine tRNA synthetase cause microcephaly and intractable epilepsy (Zhang et al. 2014). Whether similar molecular defects are seen in neurons of children with other mutations in EEF1A2 is as yet unknown. Here, EEF1A2 is linked to Ataxia.