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11 results on '"D'Agostino, Claudia"'

1. Classical BSE dismissed as the cause of CWD in Norwegian red deer despite strain similarities between both prion agents

Author

Conferencia de Rectores de las Universidades Españolas, Consejo Superior de Investigaciones Científicas (España), European Commission, Agencia Estatal de Investigación (España), Istituto Superiore di Sanità, Ministerio de Ciencia, Innovación y Universidades (España), Marín-Moreno, Alba [0000-0002-4023-6398], Benestad, Sylvie L. [0000-0002-3011-0484], Barrio, Tomás [0000-0002-7037-6316], Pirisinu, Laura [0000-0002-6548-8225], Espinosa, Juan Carlos [0000-0002-6719-9902], di Bari, Michele [0000-0003-0943-6823], Eraña, Hasier [0000-0001-8776-4211], Maddison, Ben C. [0000-0001-5453-5359], Canoyra, Sara [0000-0001-6371-8122], Castilla, Joaquín [0000-0002-2216-1361], Spiropoulos, J. [0000-0003-0119-8920], Bishop, Keith [0009-0009-8486-9486], Nonno, Romolo [0000-0001-7556-1564], Våge, Jorn [0000-0002-2016-0111], Andréoletti, Olivier [0000-0002-7369-6016], Torres, Juan María [0000-0003-0443-9232], Marín-Moreno, Alba, Benestad, Sylvie L., Barrio, Tomás, Pirisinu, Laura, Espinosa, Juan Carlos, Tran, Linh, Huor, Alvina, di Bari, Michele, Eraña, Hasier, Maddison, Ben C., D'Agostino, Claudia, Fernández-Borges, N., Canoyra, Sara, Jerez-Garrido, Nuria, Castilla, Joaquín, Spiropoulos, J., Bishop, Keith, Gough, Kevin C., Nonno, Romolo, Våge, Jorn, Andréoletti, Olivier, Torres, Juan María, Conferencia de Rectores de las Universidades Españolas, Consejo Superior de Investigaciones Científicas (España), European Commission, Agencia Estatal de Investigación (España), Istituto Superiore di Sanità, Ministerio de Ciencia, Innovación y Universidades (España), Marín-Moreno, Alba [0000-0002-4023-6398], Benestad, Sylvie L. [0000-0002-3011-0484], Barrio, Tomás [0000-0002-7037-6316], Pirisinu, Laura [0000-0002-6548-8225], Espinosa, Juan Carlos [0000-0002-6719-9902], di Bari, Michele [0000-0003-0943-6823], Eraña, Hasier [0000-0001-8776-4211], Maddison, Ben C. [0000-0001-5453-5359], Canoyra, Sara [0000-0001-6371-8122], Castilla, Joaquín [0000-0002-2216-1361], Spiropoulos, J. [0000-0003-0119-8920], Bishop, Keith [0009-0009-8486-9486], Nonno, Romolo [0000-0001-7556-1564], Våge, Jorn [0000-0002-2016-0111], Andréoletti, Olivier [0000-0002-7369-6016], Torres, Juan María [0000-0003-0443-9232], Marín-Moreno, Alba, Benestad, Sylvie L., Barrio, Tomás, Pirisinu, Laura, Espinosa, Juan Carlos, Tran, Linh, Huor, Alvina, di Bari, Michele, Eraña, Hasier, Maddison, Ben C., D'Agostino, Claudia, Fernández-Borges, N., Canoyra, Sara, Jerez-Garrido, Nuria, Castilla, Joaquín, Spiropoulos, J., Bishop, Keith, Gough, Kevin C., Nonno, Romolo, Våge, Jorn, Andréoletti, Olivier, and Torres, Juan María

Abstract

The first case of CWD in a Norwegian red deer was detected by a routine ELISA test and confirmed by western blotting and immunohistochemistry in the brain stem of the animal. Two different western blotting tests were conducted independently in two different laboratories, showing that the red deer glycoprofile was different from the Norwegian CWD reindeer and CWD moose and from North American CWD. The isolate showed nevertheless features similar to the classical BSE (BSE-C) strain. Furthermore, BSE-C could not be excluded based on the PrPSc immunohistochemistry staining in the brainstem and the absence of detectable PrPSc in the lymphoid tissues. Because of the known ability of BSE-C to cross species barriers as well as its zoonotic potential, the CWD red deer isolate was submitted to the EURL Strain Typing Expert Group (STEG) as a BSE-C suspect for further investigation. In addition, different strain typing in vivo and in vitro strategies aiming at identifying the BSE-C strain in the red deer isolate were performed independently in three research groups and BSE-C was not found in it. These results suggest that the Norwegian CWD red deer case was infected with a previously unknown CWD type and further investigation is needed to determine the characteristics of this potential new CWD strain.

Published
2024

2. Aquaporin-5 Dynamic Regulation

Author

D'Agostino, Claudia, Parisis, Dorian, Chivasso, Clara, Hajiabbas, Maryam, Soyfoo, Muhammad Shahnawaz, Delporte, Christine, D'Agostino, Claudia, Parisis, Dorian, Chivasso, Clara, Hajiabbas, Maryam, Soyfoo, Muhammad Shahnawaz, and Delporte, Christine

Abstract

info:eu-repo/semantics/published

Published
2023

3. Involvement of aquaporin 5 in Sjögren’s syndrome

Author

Chivasso, Clara, D'Agostino, Claudia, Parisis, Dorian, Soyfoo, Muhammad Shahnawaz, Delporte, Christine, Chivasso, Clara, D'Agostino, Claudia, Parisis, Dorian, Soyfoo, Muhammad Shahnawaz, and Delporte, Christine

Abstract

Sjögren's syndrome (SS) is a chronic autoimmune disease with the pathological hallmark of lymphoplasmacytic infiltration of exocrine glands - more specifically salivary and lacrimal glands - resulting in a diminished production of tears and saliva (sicca syndrome). The pathophysiology underscoring the mechanisms of the sicca symptoms in SS has still yet to be unraveled but recent advances have identified a cardinal role of aquaporin-5 (AQP5) as a key player in saliva secretion as well as salivary gland epithelial cell dysregulation. AQP5 expression and localization are significantly altered in salivary glands from patients and mice models of the disease, shedding light on a putative mechanism accounting for diminished salivary flow. Furthermore, aberrant expression and localization of AQP5 protein partners, such as prolactin-inducible protein and ezrin, may account for altered AQP5 localization in salivary glands from patients suffering from SS and are considered as new players in SS development. This review provides an overview of the role of AQP5 in SS salivary gland epithelial cell dysregulation, focusing on its trafficking and protein-protein interactions., SCOPUS: re.j, info:eu-repo/semantics/published

Published
2023

4. Bona fide atypical scrapie faithfully reproduced for the first time in a rodent model

Author

Ministerio de Economía y Competitividad (España), European Commission, Fundació La Marató de TV3, Generalitat de Catalunya, Vidal, Enric [0000-0002-4965-3286], Sánchez-Martín, Manuel A.[0000-0001-8370-1336], Eraña, Hasier [0000-0001-8776-4211], Lázaro, Sonia Pérez [0000-0002-2079-0711], Pérez-Castro, Miguel A.[0000-0003-2850-3470], Otero, Alicia [0000-0001-9075-2764], Charco, Jorge M. [0000-0003-3476-1855], Marín, Belén [0000-0002-1590-3347], López-Moreno, Rafael [0000-0002-2155-8965], Díaz-Domínguez, Carlos M. [0000-0002-0410-1963], Geijo, Mariví [0000-0002-3120-1038], Ordóñez, Montserrat [0000-0002-2389-7733], Cantero, Guillermo [0000-0003-4200-503X], di Bari, Michele [0000-0003-0943-6823], Lorenzo, Nuria L.[0000-0002-6444-6269], Pirisinu, Laura [0000-0002-6548-8225], Torres, Juan María [0000-0003-0443-9232], Béringue, V. [0000-0001-6706-5712], Telling, Glenn [0000-0002-6294-1805], Badiola, Juan J. [0000-0002-7173-7216], Pumarola, Martí [0000-0002-0935-7941], Bolea, Rosa [0000-0002-2746-3932], Nonno, Romolo [0000-0001-7556-1564], Requena, J. R. [0000-0003-1954-4975], Castilla, Joaquín [0000-0002-2216-1361], Vidal, Enric, Sánchez-Martín, Manuel Adolfo, Eraña, Hasier, Lázaro, Sonia Pérez, Pérez-Castro, Miguel A., Otero, Alicia, Charco, Jorge M., Marín, Belén, López-Moreno, Rafael, Díaz-Domínguez, Carlos M., Geijo, Mariví, Ordóñez, Montserrat, Cantero, Guillermo, di Bari, Michele, Lorenzo, Nuria L., Pirisinu, Laura, D'Agostino, Claudia, Torres, Juan María, Béringue, V., Telling, Glenn, Badiola, Juan J., Pumarola, Martí, Bolea, Rosa, Nonno, Romolo, Requena, Jesús R., Castilla, Joaquín, Ministerio de Economía y Competitividad (España), European Commission, Fundació La Marató de TV3, Generalitat de Catalunya, Vidal, Enric [0000-0002-4965-3286], Sánchez-Martín, Manuel A.[0000-0001-8370-1336], Eraña, Hasier [0000-0001-8776-4211], Lázaro, Sonia Pérez [0000-0002-2079-0711], Pérez-Castro, Miguel A.[0000-0003-2850-3470], Otero, Alicia [0000-0001-9075-2764], Charco, Jorge M. [0000-0003-3476-1855], Marín, Belén [0000-0002-1590-3347], López-Moreno, Rafael [0000-0002-2155-8965], Díaz-Domínguez, Carlos M. [0000-0002-0410-1963], Geijo, Mariví [0000-0002-3120-1038], Ordóñez, Montserrat [0000-0002-2389-7733], Cantero, Guillermo [0000-0003-4200-503X], di Bari, Michele [0000-0003-0943-6823], Lorenzo, Nuria L.[0000-0002-6444-6269], Pirisinu, Laura [0000-0002-6548-8225], Torres, Juan María [0000-0003-0443-9232], Béringue, V. [0000-0001-6706-5712], Telling, Glenn [0000-0002-6294-1805], Badiola, Juan J. [0000-0002-7173-7216], Pumarola, Martí [0000-0002-0935-7941], Bolea, Rosa [0000-0002-2746-3932], Nonno, Romolo [0000-0001-7556-1564], Requena, J. R. [0000-0003-1954-4975], Castilla, Joaquín [0000-0002-2216-1361], Vidal, Enric, Sánchez-Martín, Manuel Adolfo, Eraña, Hasier, Lázaro, Sonia Pérez, Pérez-Castro, Miguel A., Otero, Alicia, Charco, Jorge M., Marín, Belén, López-Moreno, Rafael, Díaz-Domínguez, Carlos M., Geijo, Mariví, Ordóñez, Montserrat, Cantero, Guillermo, di Bari, Michele, Lorenzo, Nuria L., Pirisinu, Laura, D'Agostino, Claudia, Torres, Juan María, Béringue, V., Telling, Glenn, Badiola, Juan J., Pumarola, Martí, Bolea, Rosa, Nonno, Romolo, Requena, Jesús R., and Castilla, Joaquín

Abstract

Atypical Scrapie, which is not linked to epidemics, is assumed to be an idiopathic spontaneous prion disease in small ruminants. Therefore, its occurrence is unlikely to be controlled through selective breeding or other strategies as it is done for classical scrapie outbreaks. Its spontaneous nature and its sporadic incidence worldwide is reminiscent of the incidence of idiopathic spontaneous prion diseases in humans, which account for more than 85% of the cases in humans. Hence, developing animal models that consistently reproduce this phenomenon of spontaneous PrP misfolding, is of importance to study the pathobiology of idiopathic spontaneous prion disorders. Transgenic mice overexpressing sheep PrPC with I112 polymorphism (TgShI112, 1-2 × PrP levels compared to sheep brain) manifest clinical signs of a spongiform encephalopathy spontaneously as early as 380 days of age. The brains of these animals show the neuropathological hallmarks of prion disease and biochemical analyses of the misfolded prion protein show a ladder-like PrPres pattern with a predominant 7-10 kDa band. Brain homogenates from spontaneously diseased transgenic mice were inoculated in several models to assess their transmissibility and characterize the prion strain generated: TgShI112 (ovine I112 ARQ PrPC), Tg338 (ovine VRQ PrPC), Tg501 (ovine ARQ PrPC), Tg340 (human M129 PrPC), Tg361 (human V129 PrPC), TgVole (bank vole I109 PrPC), bank vole (I109I PrPC), and sheep (AHQ/ARR and AHQ/AHQ churra-tensina breeds). Our analysis of the results of these bioassays concludes that the strain generated in this model is indistinguishable to that causing atypical scrapie (Nor98). Thus, we present the first faithful model for a bona fide, transmissible, ovine, atypical scrapie prion disease.

Published
2022

6. Insight into the mammalian aquaporin interactome

Author

Törnroth-Horsefield, Susanna, Chivasso, Clara, Strandberg, Helin, D'Agostino, Claudia, O'Neale, Carla V.T., Schey, Kevin L, Delporte, Christine, Törnroth-Horsefield, Susanna, Chivasso, Clara, Strandberg, Helin, D'Agostino, Claudia, O'Neale, Carla V.T., Schey, Kevin L, and Delporte, Christine

Abstract

info:eu-repo/semantics/published

Published
2022

7. Defective localization and interaction of aquaporin-5 interacting protein partners in salivary glands from patients with primary Sjögren’s syndrome

Author

International symposium on Sjögren's syndrome, D'Agostino, Claudia, Chivasso, Clara, Filipovic, Nikola, Conrard, Louise, Zindy, Egor, Perret, Jason, Soyfoo, Muhammad Shahnawaz, Moscato, Stefania, Baldini, Chiara, Mattii, Letizia, Delporte, Christine, International symposium on Sjögren's syndrome, D'Agostino, Claudia, Chivasso, Clara, Filipovic, Nikola, Conrard, Louise, Zindy, Egor, Perret, Jason, Soyfoo, Muhammad Shahnawaz, Moscato, Stefania, Baldini, Chiara, Mattii, Letizia, and Delporte, Christine

Abstract

info:eu-repo/semantics/nonPublished

Published
2022

8. Bioengineering in Salivary Gland Regeneration

Author

Hajiabbas, Maryam, D'Agostino, Claudia, Siminska-Stanny, Julia, Tran, S D, Shavandi, Amin, Delporte, Christine, Hajiabbas, Maryam, D'Agostino, Claudia, Siminska-Stanny, Julia, Tran, S D, Shavandi, Amin, and Delporte, Christine

Abstract

info:eu-repo/semantics/published

Published
2022

9. Characterization of goat prions demonstrates geographical variation of scrapie strains in Europe and reveals the composite nature of prion strains

Author

European Commission, Ministry of Agriculture, Nature and Food Quality (The Netherlands), Ministerio de Ciencia, Innovación y Universidades (España), Agencia Estatal de Investigación (España), CSIC - Instituto Nacional de Investigación y Tecnología Agraria y Alimentaria (INIA), Ministerio de Economía y Competitividad (España), Nonno, Romolo [0000-0001-7556-1564], Marín-Moreno, Alba [0000-0002-4023-6398], Espinosa Martín, Juan Carlos [0000-0002-6719-9902], Fast, C. [0000-0002-7341-7873], Van Keulen, Lucien [0000-0002-8645-5190], Spiropoulos, J. [0000-0003-0119-8920], Lantier, Isabelle [0000-0003-1250-767X], Andréoletti, Olivier [0000-0002-7369-6016], Pirisinu, Laura [0000-0002-6548-8225], di Bari, Michele [0000-0003-0943-6823], Sklaviadis, T. [0000-0002-0246-3100], Acutis, Pier Luigi [0000-0003-4814-2924], Acin, Cristina [0000-0001-5105-6133], Bossers, A. [0000-0002-6586-717X], Vaccari, Gabriel [0000-0003-0215-3521], Chiappini, Barbara [0000-0002-5260-5285], Lantier, Frederic [0000-0001-5896-330X], Groschup, M. H. [0000-0003-0215-185X], Agrimi, Umberto [0000-0001-7828-0186], Torres, Juan María [0000-0003-0443-9232], Langeveld, J. [0000-0002-4165-3230], Nonno, Romolo, Marín-Moreno, Alba, Espinosa Martín, Juan Carlos, Fast, C., Van Keulen, Lucien, Spiropoulos, J., Lantier, Isabelle, Andréoletti, Olivier, Pirisinu, Laura, di Bari, Michele, Aguilar-Calvo, Patricia, Sklaviadis, T., Papasavva-Stylianou, Penelope, Acutis, Pier Luigi, Acin, Cristina, Bossers, A., Jacobs, Jorge G., Vaccari, Gabriel, D'Agostino, Claudia, Chiappini, Barbara, Lantier, Frederic, Groschup, M. H., Agrimi, Umberto, Torres, Juan María, Langeveld, J., European Commission, Ministry of Agriculture, Nature and Food Quality (The Netherlands), Ministerio de Ciencia, Innovación y Universidades (España), Agencia Estatal de Investigación (España), CSIC - Instituto Nacional de Investigación y Tecnología Agraria y Alimentaria (INIA), Ministerio de Economía y Competitividad (España), Nonno, Romolo [0000-0001-7556-1564], Marín-Moreno, Alba [0000-0002-4023-6398], Espinosa Martín, Juan Carlos [0000-0002-6719-9902], Fast, C. [0000-0002-7341-7873], Van Keulen, Lucien [0000-0002-8645-5190], Spiropoulos, J. [0000-0003-0119-8920], Lantier, Isabelle [0000-0003-1250-767X], Andréoletti, Olivier [0000-0002-7369-6016], Pirisinu, Laura [0000-0002-6548-8225], di Bari, Michele [0000-0003-0943-6823], Sklaviadis, T. [0000-0002-0246-3100], Acutis, Pier Luigi [0000-0003-4814-2924], Acin, Cristina [0000-0001-5105-6133], Bossers, A. [0000-0002-6586-717X], Vaccari, Gabriel [0000-0003-0215-3521], Chiappini, Barbara [0000-0002-5260-5285], Lantier, Frederic [0000-0001-5896-330X], Groschup, M. H. [0000-0003-0215-185X], Agrimi, Umberto [0000-0001-7828-0186], Torres, Juan María [0000-0003-0443-9232], Langeveld, J. [0000-0002-4165-3230], Nonno, Romolo, Marín-Moreno, Alba, Espinosa Martín, Juan Carlos, Fast, C., Van Keulen, Lucien, Spiropoulos, J., Lantier, Isabelle, Andréoletti, Olivier, Pirisinu, Laura, di Bari, Michele, Aguilar-Calvo, Patricia, Sklaviadis, T., Papasavva-Stylianou, Penelope, Acutis, Pier Luigi, Acin, Cristina, Bossers, A., Jacobs, Jorge G., Vaccari, Gabriel, D'Agostino, Claudia, Chiappini, Barbara, Lantier, Frederic, Groschup, M. H., Agrimi, Umberto, Torres, Juan María, and Langeveld, J.

Abstract

Bovine Spongiform Encephalopathy (BSE) is the only animal prion which has been recognized as a zoonotic agent so far. The identification of BSE in two goats raised the need to reliably identify BSE in small ruminants. However, our understanding of scrapie strain diversity in small ruminants remains ill-defined, thus limiting the accuracy of BSE surveillance and spreading fear that BSE might lurk unrecognized in goats. We investigated prion strain diversity in a large panel of European goats by a novel experimental approach that, instead of assessing the neuropathological profile after serial transmissions in a single animal model, was based on the direct interaction of prion isolates with several recipient rodent models expressing small ruminants or heterologous prion proteins. The findings show that the biological properties of scrapie isolates display different patterns of geographical distribution in Europe and suggest that goat BSE could be reliably discriminated from a wide range of biologically and geographically diverse goat prion isolates. Finally, most field prion isolates showed composite strain features, with discrete strain components or sub-strains being present in different proportions in individual goats or tissues. This has important implications for understanding the nature and evolution of scrapie strains and their transmissibility to other species, including humans.

Published
2020

10. Insight into salivary gland aquaporins

Author

D'Agostino, Claudia, Elkashty, O, Chivasso, Clara, Perret, Jason, Tran, S D, Delporte, Christine, D'Agostino, Claudia, Elkashty, O, Chivasso, Clara, Perret, Jason, Tran, S D, and Delporte, Christine

Abstract

info:eu-repo/semantics/published

Published
2020

11. Isolation of infectious, non-fibrillar and oligomeric prions from a genetic prion disease

Author

Vanni, Ilaria; https://orcid.org/0000-0003-2617-4263, Pirisinu, Laura, Acevedo-Morantes, Claudia, Kamali-Jamil, Razieh; https://orcid.org/0000-0003-4127-0026, Rathod, Vineet, Di Bari, Michele Angelo, D’Agostino, Claudia, Marcon, Stefano, Esposito, Elena, Riccardi, Geraldina, Hornemann, Simone, Senatore, Assunta, Aguzzi, Adriano; https://orcid.org/0000-0002-0344-6708, Agrimi, Umberto, Wille, Holger, Nonno, Romolo; https://orcid.org/0000-0001-7556-1564, Vanni, Ilaria; https://orcid.org/0000-0003-2617-4263, Pirisinu, Laura, Acevedo-Morantes, Claudia, Kamali-Jamil, Razieh; https://orcid.org/0000-0003-4127-0026, Rathod, Vineet, Di Bari, Michele Angelo, D’Agostino, Claudia, Marcon, Stefano, Esposito, Elena, Riccardi, Geraldina, Hornemann, Simone, Senatore, Assunta, Aguzzi, Adriano; https://orcid.org/0000-0002-0344-6708, Agrimi, Umberto, Wille, Holger, and Nonno, Romolo; https://orcid.org/0000-0001-7556-1564

Abstract

Prions are transmissible agents causing lethal neurodegenerative diseases that are composed of aggregates of misfolded cellular prion protein (PrPSc). Despite non-fibrillar oligomers having been proposed as the most infectious prion particles, prions purified from diseased brains usually consist of large and fibrillar PrPSc aggregates, whose protease-resistant core (PrPres) encompasses the whole C-terminus of PrP. In contrast, PrPSc from Gerstmann-Sträussler-Scheinker disease associated with alanine to valine substitution at position 117 (GSS-A117V) is characterized by a small protease-resistant core, which is devoid of the C-terminus. We thus aimed to investigate the role of this unusual PrPSc in terms of infectivity, strain characteristics, and structural features. We found, by titration in bank voles, that the infectivity of GSS-A117V is extremely high (109.3 ID50 U/g) and is resistant to treatment with proteinase K (109.0 ID50 U/g). We then purified the proteinase K-resistant GSS-A117V prions and determined the amount of infectivity and PrPres in the different fractions, alongside the morphological characteristics of purified PrPres aggregates by electron microscopy. Purified pellet fractions from GSS-A117V contained the expected N- and C-terminally cleaved 7 kDa PrPres, although the yield of PrPres was low. We found that this low yield depended on the low density/small size of GSS-A117V PrPres, as it was mainly retained in the last supernatant fraction. All fractions were highly infectious, thus confirming the infectious nature of the 7 kDa PrPres, with infectivity levels that directly correlated with the PrPres amount detected. Finally, electron microscopy analysis of these fractions showed no presence of amyloid fibrils, but only very small and indistinct, non-fibrillar PrPresparticles were detected and confirmed to contain PrP via immunogold labelling. Our study demonstrates that purified aggregates of 7 kDa PrPres, spanning residues ∼90–150, are highly infe

Published
2020

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