Inactivating mutations in other mitochondrial proteases leads to developmental, metabolic, and neurological disorders such as CODAS syndrome (CODASS), Perrault syndrome 3 (PRLTS3), optic atrophy 11 (OPA11), erythropoietic protoporphyria (EPP), spinocerebellar ataxia 28 (SCA28), and hereditary spastic paraplegia 7 (HSP7) [20,28,150,151]. This evidence concerns the gene YME1L1 and spinocerebellar ataxia type 28.