What is it about?

When parents find out that their child has an incurable disease like mitochondrial disease, it is devastating. Thanks to funding from the Pacific Northwest Mitochondrial Disease Guild, we were able to further the understanding of genes that alleviate mitochondrial disease symptoms. To accomplish this, we looked to a simple worm, with lots of mitochondria used for things like feeding and swimming - in particular we experimented with worms that have the same heritable mutation that causes Leigh syndrome in humans, a mitochondrial disease. These worms don't live very long, but with the power of genetics, we can make them live even longer than they would have lived disease-free. Counter-intuitively, these worms' long life depends on molecules that would normally be associated with toxins and infection. Understanding how that works, is the topic of this paper.

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Why is it important?

We characterized a long-lived double mutant worm, to begin to uncover the "fountain of youth" that they have going. This is important because the molecular strategies they engage could potentially be manipulated to treat human mitochondrial diseases. We found that the long-lived worms still had the mitochondrial defects of their disease, so they weren't cured, they just withstand the struggle better - so much so that they withstand aging too. We determined that the effect is located in the worm's feeding muscles, concentrated in the mitochondria more than the rest of the cell, and involve antioxidants. Although this is a very early, basic stage in the research, that may or may not end up being able to extend the life of patients, the discoveries we made add to the hope that one day mitochondrial disease might be treatable.

Perspectives

At the Mito Guild, I met amazingly dedicated, loving families with children struggling to cope with their heartbreaking mitochondrial disease. I feel very privileged to have made friends and shared with them about my research on mitochondria, a topic very close to their hearts even though they are not scientists. I got to see and feel the power of science, for people who, despite their suffering, have so much hope that a cure is possible through carefully studying disease model organisms and translating that work to humans.

Dr Beverley M. (Dancy) Rabbitts
Washington State University

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This page is a summary of: Glutathione S-transferase mediates an ageing response to mitochondrial dysfunction, Mechanisms of Ageing and Development, January 2016, Elsevier, DOI: 10.1016/j.mad.2015.12.001.
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