2026-04-18T04:15:30Zhttps://recercat.cat/oai/request

oai:recercat.cat:20.500.12327/46342025-10-22T11:34:26Zcom_2072_4428com_2072_4427col_2072_487898

00925njm 22002777a 4500 dc Vidal, Enric author Eraña, Hasier author Charco, Jorge M. author Lorenzo, Nuria L. author Giler, Samanta author Ordóñez, Montserrat author Fernández-Muñoz, Eva author San-Juan-Ansoleaga, Maitena author Telling, Glenn C. author Sánchez-Martín, Manuel A. author Geijo, Mariví author Requena, Jesús R. author Castilla, Joaquín author 2025-04-11 Prion disease phenotypes (prion strains) are primarily determined by the specific misfolded conformation of the cellular prion protein (PrPC). However, post-translational modifications, including glycosyl phosphatidyl inositol (GPI) membrane anchoring and glycosylation, may influence strain characteristics. We investigated whether these modifications are essential for maintaining the unique properties of bank vole-adapted Chronic Wasting Disease (CWD-vole), the fastest known prion strain. Using a novel transgenic mouse model expressing I109 bank vole PrPC lacking the GPI anchor and largely devoid of glycans, we performed serial passages of CWD-vole prions. Despite elongated initial incubation periods, the strain maintained 100 % attack rate through three passages. Although the pathological phenotype showed characteristic GPI-less features, including abundant extracellular plaque formation, three subsequent serial passages in fully glycosylated and GPI-anchored bank vole I109 PrPC expressing transgenic mice TgVole (1×) demonstrated that the strain's distinctive rapid propagation properties were preserved. These findings suggest that neither GPI anchoring nor glycosylation are essential for maintaining CWD-vole strain properties, supporting the concept that strain characteristics are primarily encoded in the protein's misfolded structure. Vidal, Enric, Hasier Eraña, Jorge M Charco, Nuria L Lorenzo, Samanta Giler, Montserrat Ordóñez, Eva Fernández-Muñoz, et al. 2025. “Conservation of Strain Properties of Bank Vole-adapted Chronic Wasting Disease in the Absence of Glycosylation and Membrane Anchoring.” Neurobiology of Disease, 201: 106894. https://doi.org/10.1016/j.nbd.2025.106894. 0969-9961 http://hdl.handle.net/20.500.12327/4634 https://doi.org/10.1016/j.nbd.2025.106894 Conservation of strain properties of bank vole-adapted chronic wasting disease in the absence of glycosylation and membrane anchoring