Mutant prion protein enhances NMDA receptor activity, activates PKC, and triggers rapid excitotoxicity in mice.

doi:10.1172/JCI186432

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Article

Mutant prion protein enhances NMDA receptor activity, activates PKC, and triggers rapid excitotoxicity in mice.

The Journal of clinical investigation - 15 May 2025

Lin Joie, Callender Julia A, Mayfield Joshua E, McClatchy Daniel B, Ojeda-Juárez Daniel, Pourhamzeh Mahsa, Soldau Katrin, Kurt Timothy D, Danque Garrett A, Khuu Helen, Ronson Josephina E, Pizzo Donald P, Du Yixing, Gruber Maxwell A, Sevillano Alejandro M, Wang Jin, Orrú Christina D, Chen Joy, Funk Gail, Aguilar-Calvo Patricia, Aulston Brent D, Roy Subhojit, Rho Jong M, Bui Jack D, Newton Alexandra C, Lipton Stuart A, Caughey Byron, Patrick Gentry N, Doré Kim, Yates John R, Sigurdson Christina J

Abstract excerpt

Neuronal hyperexcitability precedes synapse loss in certain neurodegenerative diseases, yet the synaptic membrane interactions and downstream signaling events remain unclear. The disordered amino terminus of the prion protein (PrPC) has been implicated in aberrant signaling in prion and Alzheimer's disease. To disrupt neuronal interactions and signaling linked to the amino terminus, we CRISPR-engineered a knockin...

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PMID: 40185484
DOI: 10.1172/JCI186432
PMCID: PMC12077891

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