Table 1.

Some Mitochondrial Associated Cell Death Proteins.

Protein	Function
Bcl-2*	Anti-apoptotic, blocks Bax/Bak channel formation
Bcl-XL	Anti-apoptotic, blocks Bax/Bak channel formation
Bax*	Pro-apoptotic, forms pores for cytochrome c release
Bak*	Pro-apoptotic, forms pores for cytochrome c release
Bad	Pro-apoptotic, decoy for Bcl-2/Bcl-XL promoting Bax/Bak pore formation
Bid	Pro-apoptotic, decoy for Bcl-2/Bcl-XL promoting Bax/Bak pore formation
Noxa	Pro-apoptotic, decoy for Bcl-2/Bcl-XL promoting Bax/Bak pore formation
Puma	Pro-apoptotic, decoy for Bcl-2/Bcl-XL promoting Bax/Bak pore formation
p53*	Antagonizes activity of Bcl-2/Bcl-XL, promotes Bax/Bak oligomerization
Cytochrome c	Activator of apoptosome
Smac/DIABLO	IAP inhibitor
AIF	Antioxidant flavoprotein/released from mitochondria to promote nuclear DNA fragmentation
Endonuclease G	Released from mitochondria to promote nuclear DNA fragmentation
HtrA2/Omi	IAP inhibitor
VDAC	mPTP component in outer mitochondrial membrane
ANT+	mPTP component in inner mitochondrial membrane
Cyclophilin D+	mPTP component in mitochondrial matrix
TSPO (peripheral benzodiazepine receptor)	Modulator of mPTP
Hexokinase	Modulator of VDAC

* Changes have been reported in human ALS (see below)

⁺ A reported target of oxidative modification in mouse ALS (see below)