Adenine Nucleotide Translocase, Mitochondrial Stress, and Degenerative Cell Death

Figure 3

Schematic diagram showing the proteostatic stress model for the pathogenic mechanism of Ant1-induced degenerative cell death. This model predicts that the mutant Ant induces proteostatic stress on the mitochondrial inner membrane, which interferes with general mitochondrial biogenesis and the maintenance of membrane potential, followed by mtDNA destabilization. The severe membrane damage and mtDNA destabilization collectively contribute to degenerative cell death. Degenerative cell death is suppressed by reduced cytosolic protein synthesis which improves global proteostasis. Stars indicate the localization of mutations.