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ZAAT-mediated mitochondrial injury within the hepatocytes. (1) Hsp70 binds to ZAAT to prevent aggregation of the mutated protein targeted for proteasomal degradation. (2) Proteasomal degradation of ZAAT. (3) Interaction of the cytoplasmic chaperone with mitochondrial import machinery leading to ZAAT mitochondrial translocation. (4) Disruption of mitochondrial membrane integrity and mitochondrial dysfunction. Figure was made by BioRender.
