1. Introduction·············································································································································································································	2
2. Different Molecular Mechanisms Associated with the Same Phenotype·········································································································	2
3. Symptomatic Treatments Based on Antioxidant Cocktails································································································································	3
4. Small Molecules, Vitamins and Cofactors Administered as Single Molecules or as a Cocktail to Patients Affected by MD····················	3
4.1. Vitamin B1—Thiamine····················································································································································································	17
4.2. Vitamin B2—Riboflavin··················································································································································································	19
4.3. Vitamin B3—Niacin························································································································································································	20
4.4. Vitamin B5—Pantothenic Acid······································································································································································	21
4.5. Vitamin B6—Pyridoxine················································································································································································	21
4.6. Vitamin B7—Biotin·························································································································································································	22
4.7. Vitamin B9—Folic Acid··················································································································································································	22
4.8. Vitamin B12—Cobalamin···············································································································································································	22
4.9. Vitamin C—L-Ascorbic Acid·········································································································································································	23
4.10. Lipoic Acid····································································································································································································	23
4.11. Vitamin A—Retinol······················································································································································································	24
4.12. Vitamin D—Calciferol·················································································································································································	24
4.13. Vitamin E—α-Tocopherol···········································································································································································	24
4.14. Vitamin K—Phylloquinone··········································································································································································	25
4.15. Coenzyme Q10 (Reduced, as Ubiquinol; Oxidized, as Ubiquinone)······································································································	25
4.16. L-Carnitine/Acetyl-L-Carnitine··································································································································································	26
5. Molecular Mechanisms of ROS Production and Mitochondrial Damage Observed in Cells from Mitochondrial Patient Tissues·········	27
6. The Need for a Structural Analysis for Evaluating the Real Need for Vitamin Supplementation·····························································	27
7. Missense Mutations Responsible for Severe Encephalomyopathies: The Case of MT-ND5, NDUFAF6 and SERAC1·····························	29
8. The Case of Carnitine O-Acetyltransferase (CRAT) Deficiency·····················································································································	31
9. SLC25A10 Dicarboxylate Carrier (DIC) Deficiency··········································································································································	32
10. MDs Depending on AA-tRNA Mutations or AA-tRNA Synthetase Mutations·························································································	32
11. CoQ Analogs and Mitochondrial Delivery Systems: Organic/Inorganic Chemicals for Stimulating Mitochondrial Activity··············	37
12. Conclusions·········································································································································································································	38
Abbreviations············································································································································································································	39
References··················································································································································································································	40