Huntington's disease (HD) may be rare but among those who grew up in the '60s this disease is well known --- folk singer Woody Guthrie died at age 55 from the genetic disorder. Guthrie was the father of the well-known peace-and-love era hippie singer-songwriter Arlo Guthrie most famous for Alice's Restaurant.
Woody Guthrie inherited the progressive neurodegenerative disease from his mother and passed it on to two of his children. Their slow fatal declines were described by their caregiver as "a descent into hell." With the onset of HD a patient suffers years of physical and mental deterioration with attendant psychiatric problems leading eventually to death.
Because HD is a dominant genetic disease, children with an affected parent have a 50/50 chance of inheriting the fatal genetic disorder.
Now, 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 have shed light on why HD take so long to appear.
HD causes cells to die in specific regions of the brain. Those afflicted carry a mutated version of the protein huntingtin (Htt). Researchers knew this mutated protein played a large role in causing the disease but they did not know what kept it at bay for so many years or why, when active in later life, it attacked a specific set of brain cells, because Htt is found in every single cell in the human body.
Ferguson and Ribeiro have found receptors, responsible for communication between brain cells that interact with the mutant Htt protein and protect the brain from cell death. With time, these aging receptors, ". . . lose this compensation and the associated protective effects, which could explain the late onset of the disease."
According to the Schulich School of Medicine & Dentistry at The University of Western Ontario, "This research, funded by the Canadian Institutes of Health Research, sheds light on the onset of HD" and may pave the way "for the development of new drug therapies."
For more information on the fight against Huntington's disease, please see an earlier Digital Journal article: Study: New drug treatment for Huntington's disease shows promise