Huntington's disease (HD) is a cruel, hereditary condition that leads to severe physical and mental deterioration, psychiatric problems and eventually, death. Currently, there are no treatments to slow down or stop it. HD sufferers are born with the disease although they do not show symptoms until late in life. In a new study published in The Journal of Neuroscience, Stephen Ferguson and Fabiola Ribeiro of Robarts Research Institute at The University of Western Ontario identified a protective pathway in the brain that may explain why HD symptoms take so long to appear. The findings could also lead to new treatments for HD.
The symptoms of Huntington's disease are caused by cell death in specific regions of the brain. Patients who have HD are born with a mutated version of the protein huntingtin (Htt), which is thought to cause these toxic effects. While researchers know HD results from this single, mutated protein, no one seems to know exactly what it does, why it does not cause symptoms until later in life, or why it kills a specific set of brain cells, even though Htt is found in every single cell in the human body.