Our results could also guide research in human disease conditions as Znhit3 mutations have been reported to cause progressive encephalopathy with oedema, hypsarrhythmia, and optic atrophy (PEHO) syndrome.[38] It would be useful to explore how protein synthesis may be affected under the pathological condition caused by Znhit3 mutations and hopefully promoting protein translation could be a therapeutic modality for human patients with defective Znhit3. Here, ZNHIT3 is linked to Leber hereditary optic neuropathy.