Abnormal mitochondrial morphology and metabolic dysfunction have been observed in many
neurodegenerative disorders (NDDs). Mitochondrial dysfunction can be caused by aberrant
mitochondrial DNA, mutant nuclear proteins that interact with mitochondria directly or
indirectly, or unknown reasons. Since mitochondria play a significant role in
neurodegeneration, mitochondria- targeted therapies represent a prosperous direction for the
development of novel drug compounds that can be used to treat NDDs. This review gives a brief
description of how mitochondrial abnormalities lead to various NDDs such as Alzheimer’s
disease, Parkinson’s disease, Huntington’s disease, and amyotrophic lateral sclerosis. We further
explore the promising therapeutic effectiveness of mitochondria-directed antioxidants, MitoQ,
MitoVitE, MitoPBN, and dimebon. We have also discussed the possibility of mitochondrial gene
therapy as a therapeutic option for these NDDs.