Figures
Genetic amelioration of Huntington's disease-induced cardiac amyloidosis.
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
Citation: (2013) PLoS Genetics Issue Image | Vol. 9(12) December 2013. PLoS Genet 9(12): ev09.i12. https://doi.org/10.1371/image.pgen.v09.i12
Published: December 26, 2013
Copyright: © 2013 Melkani et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.
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