Figure 1. Mechanisms of nutritional interventions in PMDs.

At the organ level, arginine and creatine are used to improve vasodilation and energy storage. At the cellular level nutrients and metabolic modifiers and signaling activators are employed to support OXPHOS (vitamins B₃ and B₂, CoQ₁₀) and metabolic pathways (vitamin B₁, B₇, and B₉, L-carnitine, and α-lipoic acid), enhance antioxidant defenses (CoQ₁₀ and α-lipoic acid), and promote mitochondrial biogenesis (vitamin B₃, resveratrol, and arginine). Cardiolipin, dispersed throughout the mitochondrial inner membrane, is essential for optimal function of multiple metabolic enzymes. Abbreviations: CI-CV, OXPHOS complexes I-V; Q, ubiquinone; CoQ₁₀, coenzyme Q₁₀; CPT, carnitine palmitoyl transferase; cyt c, cytochrome c; MPC, mitochondrial pyruvate carrier; mtDNA, mitochondrial DNA; PDH, pyruvate dehydrogenase; ROS, reactive oxygen species, (superoxide, hydrogen peroxide and hydroxyl radical; vit, vitamin.