What Role Does Mitochondrial Stress Play in Neurodegenerative Diseases? - Protein Misfolding and Cellular Stress in Disease and Aging - page 60

Chapter 4
What Role Does Mitochondrial Stress Play
in Neurodegenerative Diseases?
Alicia Mae Pickrell and Carlos Torres Moraes
Abstract
The essential need for mitochondrial function has been extensively shown to relate to neuronal health.
Neurodegeneration and neurodegeneration-related diseases have been associated with multiple mito-
chondrial dysfunctions. This review highlights key findings related to commonly studied mitochondrial
dysfunctions: imbalance of mitochondrial dynamics, mutations in the mitochondrial genome, excessive
reactive oxygen species, and misfolded protein associations/interactions with the mitochondria. Future
research in mitochondrial function will help elucidate complex neurodegenerative events while impacting
both individual and societal health.
Key words: Mitochondrial stress, Neurodegeneration, Mitochondrial dysfunction, Reactive oxygen
species, Mitochondrial genome, Misfolded proteins
1. Introduction
Mitochondrial ATP production is vital for cellular function,
signaling pathways, and overall cell viability. This is true for all
cells; however, the reliance on proper mitochondrial function is
particularly high for neurons due to their postmitotic status,
unique electrophysiological properties, and high ATP demand.
Neurons cannot easily be replaced when dysfunctions of the mito-
chondria can no longer be compensated for. Neurodegenerative
diseases encompass an age-related regional and selective loss of
specific neural cell populations that causes behavioral and mental
decline depending on the extent and location of the cells that
were lost. Perturbations associated with mitochondria are thought
to correlate with or directly cause neurodegenerative events.