The Evolution of Experimental Rodent Models for Prion Diseases DOI
Joseph DeFranco, Glenn C. Telling

Journal of Neurochemistry, Год журнала: 2025, Номер 169(3)

Опубликована: Март 1, 2025

ABSTRACT Prion diseases are a group of fatal, neurodegenerative that affect animals and humans. These characterized by the conformational conversion normal, host‐encoded PrP C into disease‐causing prion isoform, Sc . Significant advancements in biological, genetic, research have led to capability studying this pathogenetic process using recombinant proteins, ex vivo systems, vitro models, mammalian hosts, latter being gold standard for assaying infectivity, transmission, strain evolution. While devoid nucleic acid, prions encipher information conformation their constituent infectious with diversity altering pathogenesis, host‐range dynamics, efficacy therapeutics. To properly study properties natural develop appropriate therapeutic strategies, it is essential utilize models authentically recapitulate these agents experimental hosts. In review, we examine evolution on non‐transgenic transgenic animals, primarily focusing rodent models. We discuss successes limitations each system provide insights based recent findings novel gene‐targeted mice. image

Язык: Английский

Prions in Muscles of Cervids with Chronic Wasting Disease, Norway DOI
Tram T. Vuong, Federico Angelo Cazzaniga, Linh M. Tran

и другие.

Emerging infectious diseases, Год журнала: 2025, Номер 31(2)

Опубликована: Янв. 10, 2025

Chronic wasting disease (CWD) is an emerging prion in Nordic countries and has been detected reindeer, moose, red deer since 2016. CWD sporadically moose 3 demonstrated pathologic strain characteristics different from including unexpected lack of prions outside the central nervous system as measured by standard diagnostic tests. Using protein misfolding cyclic amplification, we lymphoreticular with Norway and, remarkably, muscles both those species CWD-infected reindeer. One lymph node 1 muscle sample showed infectivity when experimentally transmitted to bank voles. Our findings highlight systemic nature strains Europe raise questions regarding risk human exposure through edible tissues.

Язык: Английский

Процитировано

0
The Evolution of Experimental Rodent Models for Prion Diseases DOI
Joseph DeFranco, Glenn C. Telling

Journal of Neurochemistry, Год журнала: 2025, Номер 169(3)

Опубликована: Март 1, 2025

ABSTRACT Prion diseases are a group of fatal, neurodegenerative that affect animals and humans. These characterized by the conformational conversion normal, host‐encoded PrP C into disease‐causing prion isoform, Sc . Significant advancements in biological, genetic, research have led to capability studying this pathogenetic process using recombinant proteins, ex vivo systems, vitro models, mammalian hosts, latter being gold standard for assaying infectivity, transmission, strain evolution. While devoid nucleic acid, prions encipher information conformation their constituent infectious with diversity altering pathogenesis, host‐range dynamics, efficacy therapeutics. To properly study properties natural develop appropriate therapeutic strategies, it is essential utilize models authentically recapitulate these agents experimental hosts. In review, we examine evolution on non‐transgenic transgenic animals, primarily focusing rodent models. We discuss successes limitations each system provide insights based recent findings novel gene‐targeted mice. image

Язык: Английский

Процитировано

0