
Frontiers in Neuroscience, Год журнала: 2024, Номер 18
Опубликована: Окт. 3, 2024
Progressive Supranuclear Palsy (PSP) is a rare and fatal neurodegenerative tauopathy which, with rapid clinical progression coupled to strong degree of clinico-pathologic correlation, has been suggested be "frontrunner" in translational development for proteinopathies. Elegant studies animals have contributed greatly our understanding disease pathogenesis PSP. However, presently no animal model replicates the key anatomical cytopathologic hallmarks, spatiotemporal spread pathology, progressive neurodegeneration, or locomotor cognitive symptoms that characterize Current models therefore likely fail recapitulate mechanisms underly pathological PSP, impeding their value. Here we review what learned about PSP from work date, examine gaps modeling discuss strategies refined will improve provide critical platform testing novel therapeutics this devastating disease.
Язык: Английский