The use of fumaric acid derivatives (I) (including their mixtures) is claimed for the production of a pharmaceutical composition for the treatment of mitochondrial diseases. - ACTIVITY : Antiparkinsonian； neuroprotective； nootropic； ophthalmological； antiinflammatory； cerebroprotective. - MECHANISM OF ACTION : Succinate dehydrogenase (SDH) stimulant. SDH is a component of mitochondrial membranes, which catalyzes the conversion of succinic acid into fumaric acid (as part of the citrate cycle) and is associated with oxidative phosphorylation, deficiency of which causes mitochondrial diseases. In tests in fibroblasts, dimethyl fumarate (Ia) at 0.75 m equivalents/l increased SDH activity 838%.
