In an analysis of 234 cases of mitochondrial nephropathy caused by pathogenic variants of genes on nDNA involved in CoQ10 synthesis, 50% of patients with pathogenic variants in COQ2 and COQ6 progressed to renal failure by 5 years of age, whereas no cases with pathogenic variants in COQ8B developed renal failure at ≤5 years of age [72]. The gene discussed is COQ6; the disease is acute kidney injury.