Huntington’s disease is associated with the accumulation of clumps of misfolded proteins in the brain and heart. These protein clusters are also linked to the deterioration of heart muscle known as cardiomyopathy, but the mechanisms by which they do the damage haven't been clear. To find out, researchers came up with a fruit fly model of Huntington’s-related protein aggregation. Using genetic manipulation techniques to ensure normal protein folding and reduce oxidative stress [harmful build-up of oxygen-containing molecules] in flies, the researchers were able to reduce the number of clusters in the heart and reverse dysfunction. It appears that protein clumps (seen here as green dots) somehow diminish and disorganize myofibrils – the rod-shaped units that make up muscle fibres (pink and cyan) – to cause heart defects. This new model system should help scientists to better understand the relationship between protein accumulation and heart muscle damage.
Written by Daniel Cossins
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