Figures & data
Figure 1. Mitochondrial ultrastructural defects in mutant PolyQ-expressing hearts are suppressed with UNC-45 and SOD overexpression. (A, A′, A″) Cardiac expression of mutant PolyQ-46 resulted in degenerated areas within the cardiomyocyte (arrow), along with fragmentation of mitochondria in cardiomyocytes. MT indicates a fragmented mitochondrion. (B, B’) Combined overexpression of SOD and UNC-45 in PolyQ-46-expressing hearts improved mitochondrial ultrastructure. MT indicates a normally shaped mitochondrion with densely packed cristae. Degeneration (arrow) was reduced to some extent in SOD+UNC-45 overexpressing hearts. VL refers to non-cardiac ventral-longitudinal fibers. Scale bar is 500 nm.
Figure 2. Mechanisms of PolyQ-induced cardiac amyloidosis. Our findings support a model in which both the oxidative stress and protein unfolding pathways underlie cardiac pathogenesis arising from mutant PolyQ. Future approaches will aim to characterize the exact molecular players associated with the production of PolyQ-induced cardiomyopathy.
