D178N prion protein mutation endows RML prions with new strain properties that do not mimic human genetic prion diseases.

doi:10.1007/s00401-026-02976-w

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D178N prion protein mutation endows RML prions with new strain properties that do not mimic human genetic prion diseases.

Acta neuropathologica - 10 Feb 2026

Masone Antonio, Grasso Anna, Comerio Liliana, Bruno Rosalia, Lavigna Giada, Vanni Ilaria, D'Agostino Claudia, Orrù Christina D, Caughey Byron, Altmeppen Hermann C, Castilla Joaquín, Giaccone Giorgio, Tagliavini Fabrizio, Di Bari Michele A, Nonno Romolo, Chiesa Roberto

Abstract excerpt

Genetic prion diseases are caused by mutant prion protein (PrP) misfolding, eventually leading to the formation of PrPSc, the infectious prion isoform that propagates by inducing misfolding of native PrP. Different mutations are thought to generate distinct prion strains with unique self-replicating and neurotoxic properties, contributing to the phenotypic diversity of genetic prion diseases. We previously showed...

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D178N prion protein mutation endows RML prions with new strain properties that do not mimic human genetic prion diseases.

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