Tze How Mok, Akin Nihat, Nour Majbour, Danielle Sequeira, Leah Holm-Mercer, Thomas Coysh, Lee Darwent, Mark Batchelor, Bradley R Groveman, Christina D Orrù, Andrew G Hughson, Amanda Heslegrave, Rhiannon Laban, Elena Veleva, Ross W Paterson, Ashvini Keshavan, Jonathan Schott, Imogen J Swift, Carolin Heller, Jonathan D Rohrer, Alexander Gerhard, Christopher Butler, James B Rowe, Mario Masellis, Miles Chapman, Michael P Lunn, Jan Bieschke, Graham S Jackson, Henrik Zetterberg, Byron Caughey, Peter Rudge, John Collinge, Simon Mead
Human prion diseases are remarkable for long incubation times followed typically by rapid clinical decline. Seed amplification assays and neurodegeneration biofluid biomarkers are remarkably useful in the clinical phase, but their potential to predict clinical onset in healthy people remains unclear. This is relevant not only to the design of preventive strategies in those at-risk of prion diseases, but more broadly, because prion-like mechanisms are thought to underpin many neurodegenerative disorders. Here, we report the accrual of a longitudinal biofluid resource in patients, controls and healthy people at risk of prion diseases, to which ultrasensitive techniques such as real-time quaking-induced conversion (RT-QuIC), and single molecule array (Simoa) digital immunoassays were applied for preclinical biomarker discovery...
March 28, 2023: Brain