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UCI MIND researchers define pathway required to slow Huntington’s disease progression in mice

Contributed by Joan Steffan, PhD

In a recent paper published in Proceedings of the National Academic of Sciences, colleagues and I show that a critical regulator of the immune system – called kinase IKKbeta – helps slow the onset of Huntington’s disease (HD) in mice, and this may be due to activation of a process called autophagy.  Autophagy helps cells clean out and recycle their ‘trash’. Accumulation of trash can occur as we age and when disease is present, so regular cleaning enabled by autophagy is critical to maintain cellular function. In HD, brain cell autophagy fails, leading to an accumulation of harmful proteins that clump together and stick to the rest of the cellular trash.  In mice, we found that kinase IKKbeta reactivates this cleaning process, so acting on this connection early might help slow progression of HD. We hope these findings help instruct the design of new therapies to treat HD, Alzheimer’s disease, and related diseases that involve age-associated accumulation of cellular trash in the brain.

Joan Steffan, PhD, is Associate Professor of Psychiatry and Human Behavior at UCI. She earned her PhD in Physiology and Pharmacology at the University of California, San Diego, first to study reproductive endocrinology, then yeast genetics, and she is now a human geneticist. Dr. Steffan is currently one of the top researchers studying Huntington’s disease. Her research focuses on neurodegeneration in Huntington’s and Alzheimer’s disease, autophagy, and the aging process.