AFG3L2 is mutated in a dominant form of SCA28 (Cagnoli et al., 2006, 2010; Di Bella et al., 2010; Smets et al., 2014), and in early‐onset recessive syndromes characterized by severe spastic‐ataxia, epilepsy, and premature death (Eskandrani et al., 2017; Pierson et al., 2011). Here, AFG3L2 is linked to spinocerebellar ataxia type 28.