2026-04-14T03:35:40Zhttps://recercat.cat/oai/request

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

Conservation of strain properties of bank vole-adapted chronic wasting disease in the absence of glycosylation and membrane anchoring Vidal, Enric Eraña, Hasier Charco, Jorge M. Lorenzo, Nuria L. Giler, Samanta Ordóñez, Montserrat Fernández-Muñoz, Eva San-Juan-Ansoleaga, Maitena Telling, Glenn C. Sánchez-Martín, Manuel A. Geijo, Mariví Requena, Jesús R. Castilla, Joaquín Producció Animal Sanitat Animal 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. 2025-10-22T11:34:26Z 2025-10-22T11:34:26Z 2025-10-22T11:34:26Z 2025-04-11 info:eu-repo/semantics/article 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 eng Neurobiology of Disease MICINN/Programa Estatal para impulsar la investigación científico-técnica y su transferencia/PID2021-122201OB-C21/ES/ANALISIS DEL MALPLEGAMIENTO IN VITRO DE UNA DIVERSIDAD DE PROTEINAS DEL PRION PARA LA GENERACION DE NUEVAS ENTIDADES INFECCIOSAS Y DESARROLLO DE APROXIMACIONES TERAPEUTICAS/ MICINN/Programa Estatal para impulsar la investigación científico-técnica y su transferencia/PID2021-122201OB-C22/ES/DESARROLLO DE INNOVADORES MODELOS DE ENFERMEDADES PRIONICAS Y ESTUDIO DE LA PATOBIOLOGIA DE PRIONES SINTETICOS EN RATONES TRANSGENICOS Y HOSPEDADORES POCO COMUNES/ EC/INTERREG-POCTEFA/EFA031-01/EU/ /NEURO-COOP ISCIII/ /AC21_2-00024/ES/Biomarcadores prodrómicos en Insomnio Familiar Letal: Un estudio longitudinal en humanos y ratones/ MICINN/Programa Estatal de generación del conocimiento y fortalecimiento científico y tecnológico del sistema I+D+I/CEX2021-001136-S/ES/ / ISCIII/ /PT23-00123/ES/ / FEDER/ / /EU/ / http://creativecommons.org/licenses/by-nc-nd/4.0/ info:eu-repo/semantics/openAccess Attribution-NonCommercial-NoDerivatives 4.0 International Elsevier