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Divergent prion strain evolution driven by PrP^C expression level in transgenic mice.

Annick Le Dur¹, Thanh Lan Laï¹, Marie-George Stinnakre²

¹Virologie Immunologie Moléculaires (VIM), INRA, Université Paris-Saclay, 78350 Jouy-en-Josas, France.

Nature Communications

|January 24, 2017

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Prion protein (PrP^C) overexpression influences prion strain evolution. High PrP^C levels in transgenic mice cause scrapie prions to change, unlike in normal conditions.

Area of Science:

Neurodegenerative diseases
Prion biology
Protein misfolding

Background:

Prions cause fatal neurodegenerative diseases by misfolding host prion protein (PrP^C) into PrP^Sc.
Distinct PrP^Sc conformers define prion strains, influencing cross-species transmission and mutation-like events.
Prion strains are typically conserved within a species.

Purpose of the Study:

To investigate how prion protein (PrP^C) gene dosage affects prion strain evolution during homotypic transmission.
To compare the strain properties of a sheep scrapie isolate in transgenic mice with varying PrP^C levels.

Main Methods:

Transmission of a sheep scrapie isolate to transgenic mouse lines expressing different levels of homologous PrP^C.

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Analysis of prion strain properties after transmission.

Main Results:

Scrapie prions bred true in mice with near-physiological PrP^C levels.
Prions evolved into different strain components in mice overexpressing PrP^C above a certain threshold.
PrP^C gene dosage influenced prion evolution during transmission within the same species.

Conclusions:

Prion protein gene dosage is a significant factor in prion strain evolution.
Overexpression of PrP^C can lead to prion adaptation and diversification.
Understanding PrP^C levels is crucial for predicting prion disease behavior.