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Dietary Schedule May Benefit Patients with Huntington Disease

A cellular process could be key to lowering levels of the Huntington disease protein.

A strict schedule of food intake may slow disease progression for patients with Huntington Disease (HD), according to a new study published by Acta Neuropathologica Communications.

The study authors found that restricting the dietary schedule of mice with HD resulted in an increased clearance rate of mutant huntingtin protein (mHTT), which is responsible for the disease.

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HD is a progressive disorder characterized by movement and psychiatric symptoms that typically appear in adults and worsen over time. Children who have a parent with HD have a 50% likelihood of developing the condition, according to the study.

The authors were able to trigger autophagy by restricting food intake in mice with HD to a 6-hour window per day. Autophagy is a biological process in which the cell cleans out debris and cellular materials, according to the authors.

Restricting food intake and increasing autophagy was linked to substantially reduced levels of mHTT in the brain of mice, according to the study.

“We know that specific aspects of autophagy don’t work properly in patients with Huntington disease,” said study lead author Dagmar Ehrnhoefer, PhD. “Our findings suggest that, at least in mice, when you fast, or eat at certain very regulated times without snacking in between meals, your body starts to increase an alternative, still functional, autophagy mechanism, which could help lower levels of the mutant huntingtin protein in the brain.”

The findings also revealed why mice expressing a mutated form of the HD gene developed no symptoms.

The modified HD gene was found to prevent the mHTT protein from being cleaved at a specific site, according to the study. These mice were observed to have a higher rate of autophagy compared with animals with regular mHTT.

Due to these findings, the authors hypothesize that the cleavage site of mHTT is crucial for controlling autophagy.

Current treatments aim to lower mHTT, but the new findings suggest that stimulating autophagy through a diet of therapeutics that target the cleavage site may be effective for HD, according to the study.

The authors noted that if confirmed in humans, small lifestyle changes could have a significant impact on those with HD.

“HD is a devastating disease with no cure available at this time,” said co-author Dale Martin, PhD. “More studies are needed, but perhaps something as simple as a modified dietary schedule could provide some benefit for patients and could be complementary to some treatments currently in clinical trials.”

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