Amyloid-like inclusions have been associated with Huntington's disease, and patients exhibit a high incidence of cardiovascular events. In this issue, Melkani and colleagues generated a Drosophila (fruit fly) model of cardiac amyloidosis. It displays accumulation of mutant Huntingtin aggregates and oxidative stress in myocardial cells upon heart-specific expression of Huntingtin protein fragments with disease-causing poly-glutamine repeats. Using genetic manipulation, the authors showed that modulation of both protein unfolding, and oxidative stress pathways, is required to ameliorate the detrimental mutant Huntingtin defects. The image shows reduced and disorganized myosin- (pink) and actin- (cyan) containing myofibrils along with mutant Huntingtin positive aggregates (green) in the heart. See Melkani et al.
Image Credit: Girish C. Melkani and colleagues
Perspectives
And Baby Makes Three: Genomic Imprinting in Plant Embryos
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PLOS Genetics: published December 5, 2013 | https://doi.org/10.1371/journal.pgen.1004009