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)