Publications

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Kortazar-Zubizarreta, I., Eraña, H., Pereda, A., Charco, J.M., Manero-Azua, A., Ruiz-Onandi, R., Aguirre, U., Gonzalez-Chinchon, G., Basque Prionopathies Study Group, Perez de Nanclares, G., Castilla, J., 2022. Analysis of a large case series of fatal familial insomnia to determine tests with the highest diagnostic value. J Neuropathol Exp Neurol 82(2):169-179. doi: 10.1093/jnen/nlac113.

Thüne K, Schmitz M, Wiedenhöft J, Shomroni O, Göbel S, Bunck T, Younas N, Zafar S, Hermann P, Zerr I. 2023 Genetic variants associated with the age of onset identified by whole-exome sequencing in fatal familial insomnia. Cells 12(16):2053. doi: 10.3390/cells12162053.

Pritzkow S, Ramirez F, Lyon A, Schulz PE, Appleby B, Moda F, Ramirez S, Notari S, Gambetti P, Soto C. 2023 Detection of prions in the urine of patients affected by sporadic Creutzfeldt-Jakob disease. Ann Clin Transl Neurol. doi: 10.1002/acn3.51919

Eraña H, Díaz-Domínguez CM, Charco JM, Vidal E, González-Miranda E, Pérez-Castro MA, Piñeiro P, López-Moreno R, Sampedro-Torres-Quevedo C, Fernández-Veiga L, Tasis-Galarza J, Lorenzo NL, Santini-Santiago A, Lázaro M, García-Martínez S, Gonçalves-Anjo N, San-Juan-Ansoleaga M, Galarza-Ahumada J, Fernández-Muñoz E, Giler S, Valle M, Telling GC, Geijó M, Requena JR, Castilla J. 2023 Understanding the key features of the spontaneous formation of bona fide prions through a novel methodology that enables their swift and consistent generation. Acta Neuropathol Commun. Sep 7;11(1):145. doi: 10.1186/s40478-023-01640-8.

Eraña H, Sampedro-Torres-Quevedo C, Charco JM, Díaz-Domínguez CM, Peccati F, San-Juan-Ansoleaga M, Vidal E, Gonçalves-Anjo N, Pérez-Castro MA, González-Miranda E, Piñeiro P, Fernández-Veiga L, Galarza-Ahumada J, Fernández-Muñoz E, Perez de Nanclares G, Telling G, Geijo M, Jiménez-Osés G, Castilla J. 2024 A Protein Misfolding Shaking Amplification-based method for the spontaneous generation of hundreds of bona fide prions.Nat Commun. 2024 Mar 8;15(1):2112. doi: 10.1038/s41467-024-46360-2.

Soliman Y, Al-khodor Y, Yildirim Koken G, Mustafaoglu N. 2024 A guide for blood–brain barrier models, FEBS Lettes. Mar;599(5):599-644. doi: 10.1002/1873-3468.15053.

Vidal E, Eraña H, Charco JM, Lorenzo NL, Giler S, Ordóñez M, Fernández-Muñoz E, San-Juan-Ansoleaga M, Telling GC, Sánchez-Martín MA, Geijo M, Requena JR, Castilla J. Conservation of strain properties of bank vole-adapted chronic wasting disease in the absence of glycosylation and membrane anchoring. Neurobiol Dis. 2025. doi: 10.1016/j.nbd.2025.106894

Sampedro-Torres-Quevedo C, Eraña H, Charco JM, Díaz-Domínguez CM, San-Juan-Ansoleaga M, Fernández-Muñoz E, Gonçalves-Anjo N, Galarza-Ahumada J, Cortaza AR, Nespolo RF, Quintero-Galvis JF, Manero-Azua A, Polanco-Alonso D, Gaite-Reguero A, Olalde I, Marigorta UM, Perez de Nanclares G, Aransay AM, Castilla J. A comprehensive phylogeny of mammalian PRNP gene reveals no influence of prion misfolding propensity on the evolution of this gene. PLoS Pathog. 2025. doi: 10.1371/journal.ppat.1013257

Díaz-Domínguez CM, Eraña H, Peccati F, Vidal E, Charco JM, Sampedro-Torres-Quevedo C, Pérez-Castro MA, Lorenzo NL, Giler S, Telling GC, Geijo M, Requena JR, Jiménez-Osés G, Castilla J. Effect of primary structural variation on cervid prion protein in flexibility, stability, and spontaneous misfolding propensity. Neurobiol Dis. 2025. doi: 10.1016/j.nbd.2025.107005

Eraña H, Vidal E, Fernández-Borges N, Charco JM, Díaz-Domínguez CM, Sampedro-Torres-Quevedo C, San-Juan-Ansoleaga M, Fernández-Muñoz E, Galarza-Ahumada J, Pérez-Castro MA, Gonçalves-Anjo N, Piñeiro P, Pirisinu L, Di Bari MA, Giler S, Raimondi I, Espinosa LC, Vanni I, D’Agostino C, Rodríguez-Cuesta L, Pasetto L, Bonetto V, González-Martín N, Teijeira S, Zou W-Q, Geijo M, Torres LM, Chiesa R, Sánchez-Martín MA, Nonno R, Requena JR, Castilla J. Overexpression of bank vole PrP(I109) in mice induces a spontaneous atypical prion disease with sex-dependent onset, early NfL elevation, and universal prion strain permissiveness. Acta Neuropathol Commun. 2026. DOI: 10.1186/s40478-025-02213-7.

Kortazar-Zubizarreta I, Manero-Azua A, Eraña H, Aguire U, Castilla J, Perez de Nanclares G. The risk of transmission of genetic prion diseases is greater than 50%. Eur J Neurol. 2025 DOI: 10.1111/ene.70455.

Fernandez Flores LC, Younas N, Goebel S, Dittmar K, Saleem T, Younas A, Budde H, Legler TJ, Möbius W, Hermann P, Schmitz M, Zerr I. Multiple antibody-coated gold nanoparticle-based ExoAssay for rapid isolation of CNS-specific exosomes from blood. Journal of Neurochemistry 2025 169, no. 10: e70263.

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