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Your search keyword '"Guiotto, Anna"' showing total 23 results
Start Over Author "Guiotto, Anna" Publisher elsevier b.v.
23 results on '"Guiotto, Anna"'

1. Current practices in clinical gait analysis in Europe: A comprehensive survey-based study from the European society for movement analysis in adults and children (ESMAC) standard initiative

Author

Armand, Stéphane, Sawacha, Zimi, Goudriaan, Marije, Horsak, Brian, van der Krogt, Marjolein, Huenaerts, Catherine, Daly, Colm, Kranzl, Andreas, Boehm, Harald, Petrarca, Maurizio, Guiotto, Anna, Merlo, Andrea, Spolaor, Fabiola, Campanini, Isabella, Cosma, Michela, Hallemans, Ann, Horemans, Herwin, Gasq, David, Moissenet, Florent, Assi, Ayman, and Sangeux, Morgan

Published
2024
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15. The constitutive activation of TLR4-IRAK1- NFκB axis is involved in the early NLRP3 inflammasome response in peripheral blood mononuclear cells of Rett syndrome patients.

Author

Cordone, Valeria, Ferrara, Francesca, Pecorelli, Alessandra, Guiotto, Anna, Vitale, Antonio, Amicarelli, Fernanda, Cervellati, Carlo, Hayek, Joussef, and Valacchi, Giuseppe

Subjects
*MONONUCLEAR leukocytes, *RETT syndrome, *NLRP3 protein, *INFLAMMASOMES, *INTERLEUKIN-1 receptors
Abstract

Rett syndrome (RTT), a devastating neurodevelopmental disorder, is caused in 95% of the cases by mutations in the X-chromosome-localized MECP2 gene. To date, RTT is considered a broad-spectrum disease, due to multisystem disturbances affecting patients, associated with mitochondrial dysfunctions, subclinical inflammation and an overall OxInflammatory status. Inflammasomes are multi-protein complexes crucially involved in innate immune responses against pathogens and oxidative stress mediators. The assembly of NLRP3:ASC inflammasome lead to pro-caspase 1 activation, maturation of interleukins (IL)-1β and 18 and proteolytic cleavage of Gasdermin D leading eventually to pyroptosis and systemic inflammation. The possible de-regulation of this system, in parallel with upstream nuclear factor (NF)-κB p65 pathway, were analyzed in peripheral blood mononuclear cells (PBMCs) and plasma isolated from RTT patients and matching controls. RTT PBMCs showed a constitutive activation of the axis TLR4 (Toll-like receptor 4)-IRAK1 (interleukin-1 receptor associated kinase 1)-NF-κB p65, together with augmented ROS generation and enhanced IL- 18 mRNA levels and NLRP3:ASC co-localization. The deregulation of inflammasome components was even found in THP-1 cells silenced for MECP2 and importantly, in plasma compartment of RTT subjects, from the earliest stages of the pathology or in correlation with the severity of MeCP2 mutations. Taken together, these data provide new insights into the mechanisms involved in RTT sub-clinical inflammatory status present in RTT patients, thus helping to reveal new targets for future therapeutic approaches. [Display omitted] • RTT PBMCs exhibit an aberrant NLRP3 inflammasome activation. • Both MeCP2 deficiency and high ROS production participate in inflammasome deregulation of RTT. • TLR4-NF-κB-IRAK1 axis is involved in IL-18 plasma levels in of RTT patients at earlier stages, suggesting a premature role of inflammasome in RTT etiopathogenesis. • RTT is a rare disease with OxInflammatory components, whose mediator is represented by constitutive activated NLRP3 inflammasome. [ABSTRACT FROM AUTHOR]

Published
2022
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17. Aberrant NF-κB / Nrf2 cross-talk in Rett syndrome.

Author

Cordone, Valeria, Pecorelli, Alessandra, Guiotto, Anna, Benedusi, Mascia, Cervellati, Carlo, Amicarelli, Fernanda, Hayek, Joussef, and Valacchi, Giuseppe

Subjects
*RETT syndrome
Abstract

Aberrant NF- B / Nrf2 cross-talk in Rett syndrome. [Extracted from the article]

Published
2021
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