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9 results on '"Scialò, Carlo"'

4. TDP-43 real-time quaking induced conversion reaction optimization and detection of seeding activity in CSF of amyotrophic lateral sclerosis and frontotemporal dementia patients

Author

Scialò, Carlo, primary, Tran, Thanh Hoa, additional, Salzano, Giulia, additional, Novi, Giovanni, additional, Caponnetto, Claudia, additional, Chiò, Adriano, additional, Calvo, Andrea, additional, Canosa, Antonio, additional, Moda, Fabio, additional, Caroppo, Paola, additional, Silani, Vincenzo, additional, Ticozzi, Nicola, additional, Ratti, Antonia, additional, Borroni, Barbara, additional, Benussi, Luisa, additional, Ghidoni, Roberta, additional, Furlanis, Giovanni, additional, Manganotti, Paolo, additional, Senigagliesi, Beatrice, additional, Parisse, Pietro, additional, Brasselet, Romain, additional, Buratti, Emanuele, additional, and Legname, Giuseppe, additional

Published
2020
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6. Comparative Analysis of C9orf72 and Sporadic Disease in a Large Multicenter ALS Population: The Effect of Male Sex on Survival of C9orf72 Positive Patients

Author

Trojsi, Francesca, primary, Siciliano, Mattia, additional, Femiano, Cinzia, additional, Santangelo, Gabriella, additional, Lunetta, Christian, additional, Calvo, Andrea, additional, Moglia, Cristina, additional, Marinou, Kalliopi, additional, Ticozzi, Nicola, additional, Ferro, Christian, additional, Scialò, Carlo, additional, Sorarù, Gianni, additional, Conte, Amelia, additional, Falzone, Yuri M., additional, Tortelli, Rosanna, additional, Russo, Massimo, additional, Sansone, Valeria Ada, additional, Chiò, Adriano, additional, Mora, Gabriele, additional, Silani, Vincenzo, additional, Volanti, Paolo, additional, Caponnetto, Claudia, additional, Querin, Giorgia, additional, Sabatelli, Mario, additional, Riva, Nilo, additional, Logroscino, Giancarlo, additional, Messina, Sonia, additional, Fasano, Antonio, additional, Monsurrò, Maria Rosaria, additional, Tedeschi, Gioacchino, additional, and Mandrioli, Jessica, additional

Published
2019
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8. Comorbidity of dementia with amyotrophic lateral sclerosis (ALS): insights from a large multicenter Italian cohort

Author

Trojsi, Francesca, Siciliano, Mattia, Femiano, Cinzia, Santangelo, Gabriella, Lunetta, Christian, Calvo, Andrea, Moglia, Cristina, Marinou, Kalliopi, Ticozzi, Nicola, Drago Ferrante, Gianluca, Scialò, Carlo, Sorarù, Gianni, Conte, Amelia, Falzone, Yuri M., Tortelli, Rosanna, Russo, Massimo, Sansone, Valeria Ada, Chiò, Adriano, Mora, Gabriele, Poletti, Barbara, Volanti, Paolo, Caponnetto, Claudia, Querin, Giorgia, Sabatelli, Mario, Riva, Nilo, Logroscino, Giancarlo, Messina, Sonia, Fasano, Antonio, Monsurrò, Maria Rosaria, Tedeschi, Gioacchino, Mandrioli, Jessica, Sabatelli, Mario (ORCID:0000-0001-6635-4985), Trojsi, Francesca, Siciliano, Mattia, Femiano, Cinzia, Santangelo, Gabriella, Lunetta, Christian, Calvo, Andrea, Moglia, Cristina, Marinou, Kalliopi, Ticozzi, Nicola, Drago Ferrante, Gianluca, Scialò, Carlo, Sorarù, Gianni, Conte, Amelia, Falzone, Yuri M., Tortelli, Rosanna, Russo, Massimo, Sansone, Valeria Ada, Chiò, Adriano, Mora, Gabriele, Poletti, Barbara, Volanti, Paolo, Caponnetto, Claudia, Querin, Giorgia, Sabatelli, Mario, Riva, Nilo, Logroscino, Giancarlo, Messina, Sonia, Fasano, Antonio, Monsurrò, Maria Rosaria, Tedeschi, Gioacchino, Mandrioli, Jessica, and Sabatelli, Mario (ORCID:0000-0001-6635-4985)

Abstract

To assess the association, at diagnosis, between amyotrophic lateral sclerosis (ALS) and dementia in a large cohort of well-characterized Italian patients. We investigated the phenotypic profile of 1638 incident patients with definite, probable or laboratory-supported probable ALS, diagnosed from January 2009 to December 2013 in 13 Italian Referral Centers, located in 10 Italian Regions, and classified in two independent subsamples accounting for presence or not of dementia. The collected ALS features, including survival and other follow-up data, were compared between the two subgroups using a one-way analysis of variance and Chi-square test, as appropriate, logistic regression models and Kaplan–Meier survival analysis. Between-subgroup comparisons showed an older age at clinical observation (p = .006), at onset and at diagnosis (p = .002) in demented versus non demented ALS patients. After adjustment for these variables, diagnosis of dementia was significantly associated with higher odds of family history of ALS (p = .001) and frontotemporal dementia (p = .003) and of bulbar onset (p = .004), and lower odds of flail leg phenotype (p = .019) and spinal onset (p = .008). The median survival time was shorter in demented versus non-demented patients, especially in case of classical, bulbar and flail limb phenotypes and both bulbar and spinal onset. Our multicenter study emphasized the importance of an early diagnosis of comorbid dementia in ALS patients, which may have clinical impact and prognostic relevance. Moreover, our results may give further inputs to validation of ALS-specific tools for the screening of cognitive impairment in clinical practice.

Published
2017

9. Prion and Prion-Like Protein Strains: Deciphering the Molecular Basis of Heterogeneity in Neurodegeneration

Author

Paolo Manganotti, Carlo Scialò, Giuseppe Legname, Elena De Cecco, Scialò, Carlo, De Cecco, Elena, Manganotti, Paolo, and Legname, Giuseppe

Subjects
0301 basic medicine, Protein Folding, Amyloid beta-Peptide, animal diseases, lcsh:QR1-502, Review, lcsh:Microbiology, Strain, prion-like proteins, Mice, 0302 clinical medicine, Settore BIO/10 - Biochimica, Infectivity, Genetics, education.field_of_study, Neurodegeneration, neurodegeneration, Neurodegenerative Diseases, DNA-Binding Proteins, Infectious Diseases, Prion, Protein folding, Human, Prions, DNA-Binding Protein, Population, Prion-like proteins, Strains, Amyloid beta-Peptides, Animals, Disease Models, Animal, Humans, Prion Proteins, tau Proteins, Context (language use), Biology, Prion Protein, DNA-binding protein, prion, strains, 03 medical and health sciences, Virology, medicine, education, Neurodegenerative Disease, Mechanism (biology), Animal, Prion-like protein, medicine.disease, nervous system diseases, 030104 developmental biology, Disease Models, Adaptation, 030217 neurology & neurosurgery
Abstract

Increasing evidence suggests that neurodegenerative disorders share a common pathogenic feature: the presence of deposits of misfolded proteins with altered physicochemical properties in the Central Nervous System. Despite a lack of infectivity, experimental data show that the replication and propagation of neurodegenerative disease-related proteins including amyloid-β (Aβ), tau, α-synuclein and the transactive response DNA-binding protein of 43 kDa (TDP-43) share a similar pathological mechanism with prions. These observations have led to the terminology of “prion-like” to distinguish between conditions with noninfectious characteristics but similarities with the prion replication and propagation process. Prions are considered to adapt their conformation to changes in the context of the environment of replication. This process is known as either prion selection or adaptation, where a distinct conformer present in the initial prion population with higher propensity to propagate in the new environment is able to prevail over the others during the replication process. In the last years, many studies have shown that prion-like proteins share not only the prion replication paradigm but also the specific ability to aggregate in different conformations, i.e., strains, with relevant clinical, diagnostic and therapeutic implications. This review focuses on the molecular basis of the strain phenomenon in prion and prion-like proteins.

Published
2019

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