 | Figure 1
Nature Medicine
10, 905 - 906 (2004)
doi:10.1038/nm0904-905
Maiming mitochondria in familial ALSValina L Dawson | | | | Figure 1. SOD-1 mutant protein gains access to mitochondria.
In two recent publications, investigators found that in familial ALS disease, mutant SOD-1 aggregates can bind to mitochondria and to the cell survival molecule Bcl-2 on mitochondria. Strikingly, mutant SOD-1 was found in the intermembrane space of mitochondria. Although the functional ramifications of this altered subcellular localization for mutant SOD-1 are not yet known, these studies raise the possibility that direct actions of the mutant SOD-1 on mitochondria diminish the cells' ability to handle stress and increase the sensitivity for cell death. TOM, translocase of the outer membrane; TIM, translocase of the inner membrane; SOD-1, superoxide dismutase-1.
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