Volume 24 Issue 6 - August 2, 2013 PDF
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Researchers Develop Disease Animal Model for Study of Frontotemporal Lobar Degeneration, a Common Cause of Dementia
Institute of Clinical Medicine, College of Medicine, National Cheng Kung University
 
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Frontotemporal lobar degeneration (FTLD) is clinically associated with atrophy in the frontal lobe and temporal lobe of the brain, memory loss, aphasia and sometimes with motor neuron disease (MND). In the over 65 age group, FTLD is the fourth most common cause of dementia after Alzheimer's disease, dementia with Lewy bodies and vascular dementia. In below-65 age group, it is the second most common cause after Alzheimer's disease. The prevalence of FTLD is 15 per 100,000 in the 45- to 64-year-old population and the mean age of onset of FTLD is 53 years. Recent literatures have shown that mismetablolism, including the elevation of the expression level, of the TAR DNA binding protein 43 (TDP-43) protein is closely correlated with a range of neurodegenerative diseases including FTLD and amyotrophic lateral sclerosis (ALS). In this study, Dr. Tsai has generated the transgenic mice overexpressing TDP-43 in the forebrain, and the mice exhibited the impaired cognition and motor performances. In addition, cells with ubiquitionted inclusions of TDP-43 in cytoplasm were detected in the diseased mouse brains. The mice were also with brain atrophy and deficits in the long-term potentiation (LTP). So far, there is no effective treatment for FTLD. The study has demonstrated the important implication that excess TDP-43 protein is sufficient to induce FTLD-U, and likely other neurodegenerative disease with TDP-43 protein as well. This principle points to a new direction for future basic and translated studies of FTLD-U. This is the first FTLD mouse model in the world and it will aid the scientists to understand the molecular mechanisms of FTLD and to develop appropriate the drugs/ treatments for the disease.
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