Cellular Models for the Study of Prions.
It is now established that numerous amyloid proteins associated with neurodegenerative diseases, including tau and α-synuclein, have essential characteristics of prions, including the ability to create transmissible cellular pathology in vivo. We have developed cellular bioassays that report on the various features of prion activity using genetic engineering and quantitative fluorescence-based detection systems. We have exploited these biosensors to measure the binding and uptake of tau seeds into cells in culture and to quantify seeding activity in brain samples. These cell models have also been used to propagate tau prion strains indefinitely in culture. In this review, we illustrate the utility of cellular biosensors to gain mechanistic insight into prion transmission and to study neurodegenerative diseases in a reductionist fashion.
Medical Scientist Training Program, Washington University School of Medicine, St. Louis, Missouri 63110.|Center for Alzheimer's and Neurodegenerative Diseases, University of Texas Southwestern Medical Center, Dallas, Texas 75390.
Brandon B
BB
Holmes
Center for Alzheimer's and Neurodegenerative Diseases, University of Texas Southwestern Medical Center, Dallas, Texas 75390.
Marc I
MI
Diamond
D000818:Animals; D015374:Biosensing Techniques; D001921:Brain / Q000378:metabolism / Q000473:pathology*; D018929:Cell Culture Techniques; D006801:Humans; D019636:Neurodegenerative Diseases / Q000209:etiology / Q000235:genetics; D017096:Prion Diseases / Q000453:epidemiology / Q000209:etiology / Q000635:transmission*; D011328:Prions / Q000235:genetics / Q000378:metabolism / Q000472:pathogenicity*; D051844:alpha-Synuclein / Q000378:metabolism; D016875:tau Proteins / Q000378:metabolism
D011328:Prions; D051844:alpha-Synuclein; D016875:tau Proteins