Norwegian moose CWD induces clinical disease and neuroinvasion in gene-targeted mice expressing cervid S138N prion protein

• Published in: PLoS pathogens Vol. 20; no. 7; p. e1012350
• Main Authors: Arifin, Maria Immaculata, Hannaoui, Samia, Ng, Raychal Ashlyn, Zeng, Doris, Zemlyankina, Irina, Ahmed-Hassan, Hanaa, Schatzl, Hermann M, Kaczmarczyk, Lech, Jackson, Walker S, Benestad, Sylvie L, Gilch, Sabine
• Format: Journal Article
• Language: English
• Published: San Francisco Public Library of Science 01.07.2024
• Subjects: Alces alces; Amino acid sequence; Animal models; Animals; Brain; Brain research; Cervus elaphus; Chronic wasting disease; Deer; Disease; Diseases; Elk; Genetically modified mice; Grants; Health aspects; Infections; Inoculum; Invasiveness; Molecular weight; Moose; Nervous system; Pathogenesis; Prion protein; Prions; Propagation; Protein seeding; Proteins; Reindeer; Spleen; Transgenic animals; Transgenic mice; Transmissible spongiform encephalopathy; Canada; Norway; North America
• ISSN: 1553-7374; 1553-7366; 1553-7374
• DOI: 10.1371/journal.ppat.1012350

Chronic wasting disease (CWD) is a prion disease affecting deer, elk and moose in North America and reindeer, moose and red deer in Northern Europe. Pathogenesis is driven by the accumulation of PrP.sup.Sc, a pathological form of the host's cellular prion protein (PrP.sup.C ), in the brain. CWD is contagious among North American cervids and Norwegian reindeer, with prions commonly found in lymphatic tissue. In Nordic moose and red deer CWD appears exclusively in older animals, and prions are confined to the CNS and undetectable in lymphatic tissues, indicating a sporadic origin.