Huntington’s Disease (HD) is a dominantly inherited genetic neurodegenerative disease. The disease is due to a mutation in a single causal gene, the Huntingtin gene, which results in an abnormal form of the protein Huntingtin. This mutation ultimately leads to the degeneration of motor neurons in the brain resulting in uncontrolled movements, loss of mental capacity and emotional disturbances.
One emotional disturbance, which has been closely associated with those affected by HD, is clinical depression. It has long been thought the depression associated with this i
s due to the fact genetic tests now allow for testing at any age, typically before onset of any symptoms, and the social reaction of knowing you have the disease. The dominant nature of the disease means enormous stress is often put on those finding out they carry the gene not only because it is known they will be affected by the disease later in life, but also because carrying the disease means having a 50% chance of passing it onto any offspring. The pedigree to the left shows the dominant nature of the heritability of Huntington's Disease.Until recently this abundance of clinical depression was thought just to be due to the knowledge of knowing one had a life-threatening disease. Research by the University of British Columbia has sought more actively for the causes of these emotional disturbances. Scientists found that mice with a defective Huntingtin gene showed signs of depression when the protein was altered. After repairing the Huntingtin protein the mice showed no more symptoms. Additionally the study explored the effectiveness of the two most common drugs, tricyclic and SSRI antidepressants, used to treat depression in people with HD and found they were ineffective at treating the depression in mice caused by the mutant Huntingtin gene. This study was based upon work by Dr. Hayden who in 2006 was able to prevent the cleavage of the Huntingtin protein in mice and as a result cure the degenerative symptoms in those mice. This study has shown much potential into one day finding a cure for the devastating HD.
Posted by Simon Yates, 41789072, Thursday P10
Reference:
http://www.physorg.com/news159200198.html
