Table 1. OXPHOS defects associated with the neurodegenerative proteinopathies discussed in this review
ProteinCIVa defectsCIb defectsMito ATP synthesis defectsΔΨmc defectsMito dynamics defectsER–mito contact alterationsMito Ca2+ handling defects
APP/Aβ++++++
α-Syn+++++++
SOD1++++++

Mito dynamics refers to the processes of fusion and fission and to mitochondrial axonal transport. ER–mito contacts refers to MAMs and functional interactions between organelles. Mito Ca2+ handling refers to mitochondrial calcium uptake and permeability transition.

  • a Complex IV.

  • b Complex I.

  • c Mitochondrial membrane potential.