Frontotemporal Dementia (FTD) is the most common cause of early-onset dementia in people under sixty years of age. It is a progressive, degenerative brain disease that gradually destroys one's ability to behave appropriately, empathize with others, learn, reason, make judgments, communicate and carry out daily activities.
FTD exhibits symptoms that are also found in other common neurodegenerative disorders. Because of this, it is often mistaken for Alzheimer's Disease, Parkinson's Disease or a primarily psychiatric illness like Bipolar disorder, depression, obsessive-compulsive disease or Schizophrenia. There is no treatment or cure yet that can reverse the damage, but medications and life changes can help relieve its symptoms.
With the help of transgenic mice, we are working to discover the underlying genetic basis of FTD and other neurodegenerative diseases like Alzheimer's and Huntington's Disease (HD). Our recent study into familial FTD have led us to discover that—contrary to the prevailing hypothesis at the time—polyglutamine expansion is not implicated in the disease.
Several trials of potential therapeutic agents have already been initiated in our YAC transgenic mouse model of HD. Novel therapeutic approaches that are effective in our HD animal model will likely have utility in treating a broad range of neurodegenerative disorders. Following the success of these trials, we plan to utilize the same approach to investigate new therapeutics in transgenic models of FTD and other neurodegenerative disorders including Amyotrophic Lateral Sclerosis and Alzheimer’s Disease.
Mackenzie IR, Butland SL, Devon RS, Dwosh E, Feldman H, Lindholm C, Neal SJ, Ouellette BF, Leavitt BR. Familial frontotemporal dementia with neuronal intranuclear inclusions is not a polyglutamine expansion disease. (2006) PMID 16945149