Interestingly, mutations in CLPP are associated with Perrault syndrome 3 (MIM 614129) which is characterised by sensorineural hearing loss and premature ovarian failure19 and CLPP knockout mice display phenotypes consistent with Perrault syndrome as well as other distinct effects such as elevated mtDNA levels and induction of selected matrix protein quality control components and inflammatory factors61. The gene discussed is CLPP; the disease is Perrault syndrome.