Researchers at The Hospital for Sick Children have made a discovery that could potentially reduce toxic cellular waste accumulation in patients with Zellweger Spectrum Disorder (ZSD). ZSD is a rare group of neurodegenerative genetic conditions characterized by a reduction in peroxisomes, vital cellular components responsible for breaking down fats.
In a study published in Nature Communications the team explains how by genetically and pharmaceutically increasing a cell’s ability to recycle its own components it is possible to clear damaged cellular material, providing a new therapeutic target for treating ZSD.
There are more than a dozen known pathways that recycle specific damaged or irrelevant components in a cell. One of these pathways is pexophagy which selectively recycles peroxisomes. Previous research found that the most common genetic variation that causes ZSD significantly increases pexophagy, causing healthy peroxisomes to get recycled alongside unhealthy ones. In the new study, it was found that this increase in pexophagy can also prevent cells from degrading other cellular waste.
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“Our work demonstrates for the first time that different cellular recycling pathways can influence one another,” senior author Kyla Germain explains. “A cell’s recycling system has a maximum load capacity—an autophagic limit. When this limit is exceeded, toxic cellular waste will accumulate.”
After locating this connection between different recycling pathways, researchers found they could optimize the overall recycling process by increasing the autophagic limit. In doing so, they observed improved clearance of cellular waste, which opens new pathways to treat ZSD.
The next phase involves testing various therapeutics in pre-clinical ZSD models to either increase autophagy or inhibit pexophagy.