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EnglishIn the last 15 years a strong correlation between oxidative stress (OxS) and Rett syndrome (RTT), a rare neurodevelopmental disorder known to be caused in 95% of the cases, by a mutation in the methyl-CpG-binding protein 2 (MECP2) gene, has been well documented. Here, we revised, summarized and discussed the current knowledge on the role of lipid peroxidation byproducts, with special emphasis on 4-hydroxynonenal (4HNE), in RTT pathophysiology. The posttranslational modifications of proteins via 4HNE, known as 4HNE protein adducts (4NHE-PAs), causing detrimental effects on protein functions, appear to contribute to the clinical severity of the syndrome, since their levels increase significantly during the subsequent 4 clinical stages, reaching the maximum degree at stage 4, represented by a late motor deterioration. In addition, 4HNE-PA are only partially removed due to the compromised functionality of the proteasome activity, contributing therefore to the cellular damage in RTT. All this will lead to a characteristic subclinical inflammation, defined “OxInflammation”, derived by a positive feedback loop between OxS byproducts and inflammatory mediators that in a long run further aggravates the clinical features of RTT patients. Therefore, in a pathology completely orphan of any therapy, aiming 4HNE as a therapeutic target could represent a coadjuvant treatment with some beneficial impact in these patients.
| Titolo: | 4-hydroxynonenal protein adducts: Key mediator in Rett syndrome oxinflammation |
| Autori: | |
| Data di pubblicazione: | 2017 |
| Rivista: | |
| Handle: | http://hdl.handle.net/11392/2362105 |
| Appare nelle tipologie: | 03.1 Articolo su rivista |
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| File | Descrizione | Tipologia | Licenza | |
|---|---|---|---|---|
| 1-s2.0-S0891584916311480-main.pdf | Post-print |  | Open Access Visualizza/Apri | |
| 1-s2.0-S0891584916311480-main.pdf | Full text (versione editoriale) | NON PUBBLICO - Accesso privato/ristretto | Administrator Richiedi una copia |
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