Drugs mentioned in the literature that may have a therapeutic effect on mitochondrial disease include coenzyme Q10, ATP, vitamins C, B1, B2, E, K1, K3, niacinamide, creatine, lipoic acid, and arginine hydrochloride [2-9]. The so-called cocktail therapy targets the final common pathway of abnormal mitochondrial function, and its drug composition and dose need long-term clinical observation, but the most commonly used are coenzyme Q10, vitamin E, and C. Most of the current studies on the efficacy of coenzyme Q10 are based on case reports or uncontrolled case studies, and the number of cases is small, the duration of dosing varies, and the drug dose is 30-300 mg/d Even high doses of coenzyme Q10 100-3000mg/d have rare side effects. Coenzyme Q10 is a naturally occurring lipid-soluble ubiquinone. Its possible therapeutic mechanism for defects in oxidative phosphorylation is the transfer of electrons from complexes 1 and 2 to complex 3, which is coupled to the synthesis of ATP. Its reduced form, ubiquinol, inhibits lipid peroxidation and protects proteins and DNA of the inner mitochondrial membrane from oxidative damage. Coenzyme Q10 has been found to improve clinical symptoms in patients with a range of oxidative phosphorylation defects, including improvements in cardiac conduction defects and eye movement abnormalities, reductions in muscle weakness and exercise intolerance, and improvements in biochemical markers such as lactate and pyruvate. A 6-month treatment period found that coenzyme Q10 improved oxidative phosphorylation and increased ATP synthesis. However, large randomized double-blind controlled clinical trials are lacking. Despite the importance of coenzyme Q10 for cellular metabolism, there has been only one clinical report of coenzyme Q10 deficiency to date. Most coenzyme Q10 deficiencies are secondary to other causes. Mitochondrial encephalomyopathy due to primary coenzyme Q10 deficiency in muscle is characterized by visible fragmentation of red fibers and lipid deposits in muscle, recurrent myoglobinuria, seizures, ataxia and mental retardation. These symptoms are significantly improved with Coenzyme Q10 in this group of patients. Vitamin C, as a reducing agent, has antioxidant effects and reduces oxygen radical damage. Combined with vitamin K3, it can directly provide electrons to cytochrome C. It can effectively improve the symptoms of patients with complex 3 deficiency, and can also be used in patients with other oxidative phosphorylation defects. Riboflavin (vitamin B2) is a precursor of FMN and FAD and can be used as a cofactor for complexes 1 and 2. Abnormalities in mitochondrial biochemical function and morphology are found in riboflavin-deficient rats. Riboflavin has been shown to improve symptoms in patients with complex 1 deficiency. It is particularly effective in the myopathic form of complex 1 deficiency, with improved muscle strength, reduced exercise intolerance, and increased enzyme activity in complex 1 after treatment. The dose of vitamin B2 for the treatment of oxidative phosphorylation defects is 30-300 mg/d. Vitamin B1 is a coenzyme in the oxidative decarboxylation of pyruvate and a-ketoglutarate. It may enhance aerobic glycolysis by enhancing pyruvate decarboxylation, thereby reducing blood lactate and pyruvate levels. Two cases of familial vitamin B1 deficiency, manifesting as myopathy with mutations at the mtDNA3243 locus, have been reported, and symptoms improved with vitamin B1 supplementation. In patients with oxidative phosphorylation deficiency vitamin B1 is often combined with other cofactors at doses of 25-300 mg/d. Vitamin E scavenges free radicals and prevents lipid peroxidation, thereby maintaining cell membrane integrity. Free radical production is increased in complex 1 functional impairment and the tocopherol to cholesterol ratio in affected tissues is reduced, reflecting increased consumption of tocopherols. Several studies have shown greater efficacy of vitamin E in combination with coenzyme Q10. However, the efficacy of vitamin E in mitochondrial disease needs further confirmation. In conclusion, oral vitamins and cofactors are convenient, inexpensive and have few side effects. Based on the lack of specific treatment for mitochondrial diseases, the combination of vitamins and cofactors is not a bad treatment for mitochondrial diseases.