Cellular housekeeping process implicated in fatal neurological disorder

Washington University School of Medicine in St. Louis • October 28, 2022

A study from Washington University School of Medicine in St. Louis shows that as patients age, Huntington's disease impairs autophagy, which eliminates waste from cells. Shown at left are neurons transformed from skin cells of a young patient with pre-symptomatic Huntington's disease. On the right are neurons transformed from skin cells of an older patient with symptomatic Huntington's; these cells are sparse because the aging process impairs autophagy, leading to cell death.

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.