Prions, the infectious agents that cause the lethal brain disease Creutzfeldt-Jakob disease (CJD) in humans infect a range of different cells in the brain and in the periphery. In contrast to other infectious diseases, like viral or bacterial diseases, prions are not recognised as rogue proteins by the immune system and therefore expand rapidly and unhindered.
We seek to better understand how prions replicate in neuronal cells to identify novel ways for therapeutic intervention. Engineering of prion-susceptible cell models enables us to scrutinise the molecular underpinnings of protein misfolding. This helps us to identify gene regulatory networks that are associated with prion replication and secretion. Identification of antibodies that specifically recognise disease-associated forms of the prion protein in our laboratory will greatly support our endeavour to investigate prion replication in vivo and in vitro
To investigate what gene-encoded proteins contribute to prion propagation we establish cell models and diagnostic assays. The prion protein (PrP) can be visualised in healthy and prion-infected cells with antisera against PrP (panel A). While PrP decorates the membranes in control cells, aggregates of misfolded PrP are apparent in the periphery of infected cells. The Scrapie Cell Assay helps us to quantify the degree of prion infection (panel B).
Philiastides A , Ribes JM , Yip DC , Schmidt C , Benilova I , Klöhn PC
Viruses. 2019 Sep 22;11(10). pii: E888. doi: 10.3390/v11100888.
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