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TITLE Establishment of Frontotemporal Dementia Patient-Specific Induced Pluripotent Stem (iPS) Cell Lines with Defined Genetic Mutations ABSTRACT We propose to generate induced pluripotent stem (iPS) cells from skin cells derived from human subjects with frontotemporal dementia (FTD). FTD accounts for 1520% of all dementia cases and, with newly identified genetic causes, is now recognized as the most common dementia in patients under 65 years of age. FTD patients suffer progressive neurodegeneration in the frontal and temporal lobes and other brain regions, resulting in behavioral changes and memory and motor neuron deficits. The median age of onset for this devastating disease is 58 years, and disease progression is rapid, with death in 38 years. Compared with other age-dependent neurodegenerative diseases, the molecular, cellular, and genetic bases of FTD remain poorly understood. Genetic causes are estimated to account for ~40% of FTD. In addition to tau identified in 1998, mutations in three causative genes have been identified during the last three years. The identification of FTD mutations opens exciting new avenues for understanding the causes of FTD. Research on these mutations will help to identify effective therapies. However, the ability to study the functions of these factors is severely limited due to the lack of avail
PI Fen-Biao Gao INSTITUTE The J. David Gladstone Institutes STATE California AMOUNT $1,708,560.00 AWARD DATE 2008 June GRANT TYPE New Cell Lines