Similarly to HIGM2, long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency, mitochondrial recessive ataxia syndrome (MIRAS) and a myopathic type of mtDNA depletion syndrome (TK2 deficiency) are caused by ancestral variants more prevalent in the Finnish population and thus share many features with FDH diseases. Here, AICDA is linked to recessive mitochondrial ataxia syndrome.