ABSTRACT
MtDNA encodes several genes essential for energy production, and in a strange quirk of nature these genes appear to hold life and death powers over the human organism-acting as a master pacemaker for aging and brain span (Kukat et al., 2011; Amati-Bonneau et al., 2008; Seo et al., 2010; Nakada et al., 2009; Paradies et al., 2011). In this chapter we explore how replication errors of mtDNA are linked to energy stress and aging in mice and neurodegeneration in humans. A mitochondrial, fusion-based mechanism pushing back against energy stress is also discussed. Interestingly, a genetically engineered mutator gene was found to accelerate aging in mice and occurs naturally in humans (see Chapter 8), where it causes premature death of neurons. This suggests that energy stress can strike the brain selectively, in essence fast-forwarding aging of the brain.
