Huntington’s disease, a fatal, inherited neurodegenerative condition, is caused by a genetic error present at birth, though its symptoms often don’t begin until middle adulthood. Scientists at Washington University School of Medicine in St. Louis have been trying to understand how the aging process triggers the onset of symptoms, with the expectation that such knowledge could point to treatments that delay or prevent neurodegeneration.
To that end, a new study from Washington University indicates that as patients age, the disease gradually impairs an important cellular housekeeping process called autophagy, which is responsible for eliminating waste from cells. This housekeeping is significant in Huntington’s because a buildup of waste in a specific kind of neuron leads to such cells’ untimely deaths.
The researchers also showed that enhancing the autophagy pathway in such neurons that were created from skin cells of Huntington’s patients protects those cells from dying.
“Our study reveals how aging triggers a loss of the crucial process of autophagy — and hints at how we might try to restore this important function, with the aim of delaying or even preventing Huntington’s disease,” said senior author Andrew S. Yoo, PhD, a Washington University professor of developmental biology.
The study, published Oct. 27 in the journal Nature Neuroscience, also may offer clues to understanding cognitive decline in aging generally.
Huntington’s disease destroys a specific type of brain cell called medium spiny neurons, the loss of which causes involuntary muscle movements, impaired mental health and cognitive decline. Patients typically live about 20 years after signs of the disease first appear.
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