Výsledky vyhledávání - "Luigi Celauro"

Akademický článek

Transmission of Norwegian reindeer CWD to sheep by intracerebral inoculation results in an unusual phenotype and prion distribution

Autor: Erez Harpaz, Federico Angelo Cazzaniga, Linh Tran, Tram T. Vuong, Giuseppe Bufano, Øyvind Salvesen, Maiken Gravdal, Devin Aldaz, Julianna Sun, Sehun Kim, Luigi Celauro, Giuseppe Legname, Glenn C. Telling, Michael A. Tranulis, Sylvie L. Benestad, Arild Espenes, Fabio Moda, Cecilie Ersdal

Publikováno v: Veterinary Research, Vol 55, Iss 1, Pp 1-13 (2024)

Abstract Chronic wasting disease (CWD), a prion disease affecting cervids, has been known in North America (NA) since the 1960s and emerged in Norway in 2016. Surveillance and studies have revealed that there are different forms of CWD in Fennoscandi

Externí odkaz: https://doaj.org/article/40a88803814242428dcf491c617529ef

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Akademický článek

Tau seeding activity in skin biopsy differentiates tauopathies from synucleinopathies

Publikováno v: npj Parkinson's Disease, Vol 10, Iss 1, Pp 1-12 (2024)

Abstract Most neurodegenerative diseases lack definitive diagnostic tests, and the identification of easily accessible and reliable biomarkers remains a critical unmet need. Since tau protein is highly expressed in skin of tauopathies patients, we ai

Externí odkaz: https://doaj.org/article/d2c75e73c14a4340ad7b432625a58ce8

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Akademický článek

PMCA-Based Detection of Prions in the Olfactory Mucosa of Patients With Sporadic Creutzfeldt–Jakob Disease

Publikováno v: Frontiers in Aging Neuroscience, Vol 14 (2022)

Sporadic Creutzfeldt-Jakob disease (sCJD) is a rare neurodegenerative disorder caused by the conformational conversion of the prion protein (PrPC) into an abnormally folded form, named prion (or PrPSc). The combination of the polymorphism at codon 12

Externí odkaz: https://doaj.org/article/4a98baad86204ee3be173b36f764ec46

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Akademický článek

Histone Acetylation Defects in Brain Precursor Cells: A Potential Pathogenic Mechanism Causing Proliferation and Differentiation Dysfunctions in Mitochondrial Aspartate-Glutamate Carrier Isoform 1 Deficiency

Autor: Eleonora Poeta, Sabrina Petralla, Giorgia Babini, Brunaldo Renzi, Luigi Celauro, Maria Chiara Magnifico, Simona Nicole Barile, Martina Masotti, Francesca De Chirico, Francesca Massenzio, Luigi Viggiano, Luigi Palmieri, Marco Virgili, Francesco Massimo Lasorsa, Barbara Monti

Publikováno v: Frontiers in Cellular Neuroscience, Vol 15 (2022)

Mitochondrial aspartate-glutamate carrier isoform 1 (AGC1) deficiency is an ultra-rare genetic disease characterized by global hypomyelination and brain atrophy, caused by mutations in the SLC25A12 gene leading to a reduction in AGC1 activity. In bot

Externí odkaz: https://doaj.org/article/3c826b154e7040839093fa65e45936ff

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Akademický článek

PMCA-generated prions from the olfactory mucosa of patients with Fatal Familial Insomnia cause prion disease in mice

Autor: Edoardo Bistaffa, Alba Marín-Moreno, Juan Carlos Espinosa, Chiara Maria Giulia De Luca, Federico Angelo Cazzaniga, Sara Maria Portaleone, Luigi Celauro, Giuseppe Legname, Giorgio Giaccone, Juan Maria Torres, Fabio Moda

Publikováno v: eLife, Vol 10 (2021)

Background: Fatal Familial Insomnia (FFI) is a genetic prion disease caused by the D178N mutation in the prion protein gene (PRNP) in coupling phase with methionine at PRNP 129. In 2017, we have shown that the olfactory mucosa (OM) collected from FFI

Externí odkaz: https://doaj.org/article/4644b327b7314f66a695129f90b90075

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Akademický článek

The Alpha-Synuclein RT-QuIC Products Generated by the Olfactory Mucosa of Patients with Parkinson’s Disease and Multiple System Atrophy Induce Inflammatory Responses in SH-SY5Y Cells

Autor: Chiara Maria Giulia De Luca, Alessandra Consonni, Federico Angelo Cazzaniga, Edoardo Bistaffa, Giuseppe Bufano, Giorgia Quitarrini, Luigi Celauro, Giuseppe Legname, Roberto Eleopra, Fulvio Baggi, Giorgio Giaccone, Fabio Moda

Publikováno v: Cells, Vol 11, Iss 1, p 87 (2021)

Parkinson’s disease (PD) and multiple system atrophy (MSA) are caused by two distinct strains of disease-associated α-synuclein (αSynD). Recently, we have shown that olfactory mucosa (OM) samples of patients with PD and MSA can seed the aggregati

Externí odkaz: https://doaj.org/article/c24dc821d151413ba546136a726e273e

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Akademický článek

The Cellular Prion Protein Increases the Uptake and Toxicity of TDP-43 Fibrils

Autor: Carlo Scialò, Luigi Celauro, Marco Zattoni, Thanh Hoa Tran, Edoardo Bistaffa, Fabio Moda, Robert Kammerer, Emanuele Buratti, Giuseppe Legname

Publikováno v: Viruses, Vol 13, Iss 8, p 1625 (2021)

Cytoplasmic aggregation of the primarily nuclear TAR DNA-binding protein 43 (TDP-43) affects neurons in most amyotrophic lateral sclerosis (ALS) and approximately half of frontotemporal lobar degeneration (FTLD) cases. The cellular prion protein, PrP

Externí odkaz: https://doaj.org/article/5bd8b303b674454e88a574e3d92f347a

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Structural and dynamical determinants of a β-sheet-enriched intermediate involved in amyloid fibrillar assembly of human prion protein

Autor: Luigi Russo, Giulia Salzano, Andrea Corvino, Edoardo Bistaffa, Fabio Moda, Luigi Celauro, Gianluca D'Abrosca, Carla Isernia, Danilo Milardi, Gabriele Giachin, Gaetano Malgieri, Giuseppe Legname, Roberto Fattorusso

Publikováno v: Chemical Science. 13:10406-10427

The N-ter domain in HuPrP regulates the folding mechanism by tuning the long-range μs–ms dynamics. Removal of the N-ter domain triggers the formation of a stable β-enriched intermediate state inducing amyloid aggregates with HuPrPSc seeding activ

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::cd0c8cb5f4b03d1dbf617fc0483a2642
https://doi.org/10.1039/d2sc00345g

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