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  
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.

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