Mitochondrial encephalomyopathies: therapeutic approaches

Therapy of mitochondrial encephalomyopathies (defined restrictively as defe cts of the mitochondrial respiratory chain) is woefully inadequate, despite great progress in our understanding of the molecular bases of these disord ers. We review available and experimental therapeutic approaches, which fal l into seven categories: (1) palliative therapy; (2) removal of noxious met abolites; (3) administration of artificial electron accepters; (4) administ ration of metabolites and cofactors; (5) administration of oxygen radical s cavengers; (6) gene therapy; and (7) genetic counseling. Progress in each o f these approaches provides some glimmer of hope for the future, although m uch work remains to be done.