Mutations in the mitochondrial protease CLPP result in Perrault syndrome type 3 (PRLTS3), an autosomal recessive disorder characterized by complete infertility due to female primary ovarian insufficiency and male azoospermia after meiotic arrest at diplotene, with sensorineural deafness, ataxia, leukodystrophy, and neuropathy [11,12,13]. Here, CLPP is linked to Perrault syndrome.