Table 6.

Proteins involved in mitochondrial dysfunction in ALS.

Molecule	Function	Ref.
SOD-1	Its aggregation impairs proper mitophagy by optineurin sequestration, and axonal transport of mitochondria	[345]
TDP-43	Involved in interaction with mitochondrial proteins	[349]
TIM22	TDP-43 gains access to the mitochondrial matrix via the mitochondrial import inner membrane	[350]
CHCHD10	Involved in mitochondrial defects	[337]
Dbr1	suppresses TDP-43 toxicity	[351]
Ataxin 2	Increased risk for ALS	[352]
GSK-3β	disrupt the VAPB PTPIP51 interaction and ER–mitochondria associations	[353]
Alsin	Increased susceptibility to oxidative stress due to a defect in Rab5 relocation to mitochondria	[354]