Antioxidant-rich, Ketogenic Diet for Pompe Mice Boosted ERT Benefits

A diet combining an oral ketone precursor — a molecule being studied as a dietary supplement — and a cocktail of natural antioxidant molecules boosts the efficacy of enzyme replacement therapy (ERT) at maintaining muscle strength and motor function in a mouse model of Pompe disease, a study shows.

These benefits were associated with improvements in muscle cells’ autophagy and oxidative stress — two cellular processes implicated in Pompe.

A ketogenic diet — one low in carbohydrates and high in fat that form an alternative energy source called ketones — did not provide the same molecular or functional benefits as diets containing the ketone precursor.

These findings suggest that the ketone precursor may be superior to a ketogenic diet at improving ERT efficacy and that combining it with an antioxidant cocktail may provide even greater benefits in terms of muscle health.

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Still, more studies are needed to assess the safety and tolerability of long-term consumption of ketone precursor supplements in patients, the researchers noted.

The study, “Nutritional co-therapy with 1,3-butanediol and multi-ingredient antioxidants enhances autophagic clearance in Pompe disease,” was published in the journal Molecular Genetics and Metabolism.

Pompe disease is caused by mutations in the GAA gene, resulting in low-to-no levels of functional acid alpha-glucosidase (GAA), an enzyme that breaks down a large sugar molecule called glycogen into glucose — the body’s go-to energy source.

GAA deficiency leads to the toxic buildup of glycogen inside small recycling structures of cells called lysosomes and negatively affects autophagy and mitochondrial function, while increasing oxidative stress.

Autophagy is a process by which the cells clean out damaged or unnecessary components, while mitochondria are the cells’ powerhouses, using glucose, fatty molecules, and, in certain circumstances, ketone bodies to produce energy.

Oxidative stress is a type of cellular damage resulting from an imbalance between the production of potentially harmful oxidant molecules (mainly derived from mitochondria’s…

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