Mitochondrial Quality Control: Decommissioning Power Plants in Neurodegenerative Diseases

Figure 1

Mitochondrial changes in neurodegeneration. Most neurodegenerative diseases result in defects in oxidative phosphorylation, respiratory dysfunction, increased ROS production, lowered mitochondrial membrane potential, decrease in synthesis of antioxidants, mtDNA mutations, impaired protein import, increased fragmentation of mitochondria, and activation of mitophagy and apoptosis. Sources of reactive oxygen species are marked by red stars. Abbreviations: Htt, Huntingtin; Ub, ubiquitin; ΔΨ, membrane potential; MPTP, 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine; ROS, reactive oxygen species; mtDNA, mitochondrial DNA; Aβ, amyloid beta; APP, amyloid precursor protein; TIM, translocase of inner membrane; TOM, translocase of outer membrane; SOD1, superoxide dismutase 1; VDAC, voltage dependent anion channel; ANT, adenine nucleotide translocator; Pink1, PTEN-induced putative kinase 1; αKGDH, alpha ketoglutarate dehydrogenase; cyt c, cytochrome c.