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Preclinical Models
Published in George C. Kagadis, Nancy L. Ford, Dimitrios N. Karnabatidis, George K. Loudos, Handbook of Small Animal Imaging, 2018
Irene Cuadrado, Jesús Egido, Jose Luis Zamorano, Carlos Zaragoza
Dementia is a pathological age-related condition, progressive and yet incurable. Common forms of dementia include Alzheimer’s disease, semantic dementia, or frontotemporal dementia, featuring a frontotemporal lobar degeneration (FTLD) of the brain. FTLD-tau is a form of FTLD in which the microtubule-associated protein tau (MAPT) does not correctly polymerize (Goedert et al. 1996). Transgenic mice overexpressing different mutated forms of FTLD-tau were found useful at the molecular level to characterize intracellular signals triggered during the progression of disease (Goedert et al. 1998; Probst et al. 2000).
The New Zealand Genetic Frontotemporal Dementia Study (FTDGeNZ): a longitudinal study of pre-symptomatic biomarkers
Published in Journal of the Royal Society of New Zealand, 2023
Brigid Ryan, Ashleigh O’Mara Baker, Christina Ilse, Kiri L. Brickell, Hannah M. Kersten, Joanna M. Williams, Donna Rose Addis, Lynette J. Tippett, Maurice A. Curtis
Pathologically, FTD is characterised by atrophy of cortical grey matter and axons in the frontal and/or anterior temporal lobes, known as frontotemporal lobar degeneration (FTLD), accompanied by neuronal and/or glial inclusion of abnormally folded proteins (Borroni et al. 2019). FTLD subtypes are categorised according to the pathological protein, which is either TAR DNA binding protein 43 (FTLD-TDP; (Mackenzie et al. 2011)); microtubule-associated protein tau (FTLD-Tau; (Dickson et al. 2011)); or RNA-binding protein fused in sarcoma (FTLD-FUS; (Neumann et al. 2009)). Very rarely, inclusions are positive for ubiquitin but negative for TDP-43, tau, and FUS; these cases are known as FTLD-UPS.