New research findings by Western Australian scientists have discovered that a communication breakdown may be responsible for causing heart failure in people with muscular dystrophy.
|Professor Aleksandra Filipovska|
Award winning mitochondrial scientist, Professor Aleksandra Filipovska, from the Harry Perkins Institute of Medical Research was among the team making the discovery which has been published in the Proceedings of National Academy of Sciences USA.
Professor Filipovska said that the work underscored the importance of mitochondria in heart disease.
According to the study, mice that lacked the protein dystrophin had impaired functional communication between a calcium channel in the muscle cell membrane and mitochondria that are responsible for producing most of the cells’ energy.
It’s understood that the hearts of muscular dystrophy patients fail but the mechanisms are unclear. The UWA team led by Professor Livia Hool have identified a link between the channel and mitochondria that involves dystrophin.
“This study demonstrated that when dystrophin is absent and the cell structure is in disarray, the communication between the channel and mitochondria becomes disrupted leading to heart failure,” said lead author, Professor Livia Hool, from the University of WA’s School of Anatomy, Physiology and Human Biology.
“This is an important finding, because it provides insight into a mechanism for the development of heart failure in muscular dystrophy,” Professor Hool said.
The ground-breaking research is funded by the National Health and Medical Research Council of Australia, Australian Research Council of Australia, Heart Foundation of Australia, National Institutes of Health USA and Muscular Dystrophy Association USA. The collaborative group also holds NHMRC Project Grants to further improve uptake of the oligomer peptides into the heart so that therapy can be optimised in patients with Duchenne Muscular Dystrophy.