Abstract: Mitochondria dysfunction occurs in the aging brain as well as in several neurodegenerative disorders and predisposes neuronal cells to enhanced sensitivity to neurotoxins. In particular, defects in any of the mitochondria respiratory chain complexes lead to impaired adenosine triphosphate production resulting in diseases that often affect the central nervous system. For instance, innate deficits in succinate dehydrogenase (SDH) mitochondria respiratory chain complex II activity caused by genetic mutations in SDH subunits lead to early-onset neurodegeneration (Jain-Ghai et al., 2013), while several adult-onset genetic neurodegenerative disorders are associated with variable levels of complex II deficiency in the central nervous system (Túnez et al., 2010). Also, chemically induced complex II deficiency leads to neurodegeneration. Livestock and human poisoning by plants or fungi containing 3-nitropropionic acid (3-NP), a naturally occurring neurotoxin that irreversibly inhibits complex II SDH activity, leads to impaired mitochondrial bioenergetics, oxidative stress, and loss of adenosine triphosphate, triggering a cascade of intracellular events that ultimately result in neuronal cell death (Túnez et al., 2010). Interestingly, in all cases, whether genetic or chemically induced, despite similar reduction in SDH activity throughout the central nervous system, neuronal degeneration is restricted to the basal ganglia, with the striatum being particularly susceptible (Túnez et al., 2010; Jain-Ghai et al., 2013).