For example, the Pdss2 deficiency mouse model exhibits autoimmune kidney disease20; mice lacking Coq7 suffer hepatic impairment of respiratory chain function21; the Coq8a (Adck3) null model develops a slowly progressive cerebellar ataxia22; and Coq9 knockouts display encephalopathy.23 The gene discussed is COQ8A; the disease is Encephalopathy.