ArticleRedox biology2020
Altered inflammasome machinery as a key player in the perpetuation of Rett syndrome oxinflammation.
Article in Redox biology, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 17 papers, 1 of them a synthesis that pooled it.
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Who cites it
17 citing papers in PubMed, 1 synthesis or guideline pooled it, 43 citations in OpenAlex.
- Movement disorders in patients with Rett syndrome: A systematic review of evidence and associated clinical considerations.Psychiatry and clinical neurosciences · 2021Pooled it
- Deregulated Nrf2-Keap1-BACH1 axis in autism spectrum disorder.Redox biology · 2025Article
- Review
- p75NTR Modulation Reduces Oxidative Stress and the Expression of Pro-Inflammatory Mediators in a Cell Model of Rett Syndrome.Biomedicines · 2024Article
- Comprehensive High-Depth Proteomic Analysis of Plasma Extracellular Vesicles Containing Preparations in Rett Syndrome.Biomedicines · 2024Article
- Role of immune cell interactions in alcohol-associated liver diseases.Liver research (Beijing, China) · 2024Review
- Dental Pulp Stem Cells Modulate Inflammasome Pathway and Collagen Deposition of Dermal Fibroblasts.Cells · 2024Article
- Gut microbiota profile in CDKL5 deficiency disorder patients.Scientific reports · 2024Article
- Article
- Rett and Rett-related disorders: Common mechanisms for shared symptoms?Experimental biology and medicine (Maywood, N.J.) · 2023Review
- Pyroptosis in Periprosthetic Osteolysis.Biomolecules · 2022Review
- Extra Virgin Olive Oil Extracts of Indigenous Southern Tuscany Cultivar Act as Anti-Inflammatory and Vasorelaxant Nutraceuticals.Antioxidants (Basel, Switzerland) · 2022Article
- MicroRNA Alterations Induced in Human Skin by Diesel Fumes, Ozone, and UV Radiation.Journal of personalized medicine · 2022Article
- TI: NLRP3 Inflammasome-Dependent Pyroptosis in CNS Trauma: A Potential Therapeutic Target.Frontiers in cell and developmental biology · 2022Review
- MeCP2 deficiency exacerbates the neuroinflammatory setting and autoreactive response during an autoimmune challenge.Scientific reports · 2021Article
- Identification of a De Novo Xq26.2 Microduplication EncompassingDiagnostics (Basel, Switzerland) · 2020Article
- The MeCP2E1/E2-BDNF-Frontiers in cell and developmental biology · 2020Article
Corrections and comments
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Authors and funding
8 authors at 5 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Rett syndrome (RTT) is a progressive neurodevelopmental disorder mainly caused by mutations in the X-linked MECP2 gene. RTT patients show multisystem disturbances associated with an oxinflammatory status. Inflammasomes are multi-protein complexes, responsible for host immune responses against pathogen infections and redox-related cellular stress. Assembly of NLRP3/ASC inflammasome triggers pro-caspase-1 activation, thus, resulting in IL-1β and IL-18 maturation. However, an aberrant activation of inflammasome system has been implicated in several human diseases. Our aim was to investigate the possible role of inflammasome in the chronic subclinical inflammatory condition typical of RTT, by analyzing this complex in basal and lipopolysaccharide (LPS)+ATP-stimulated primary fibroblasts, as well as in serum from RTT patients and healthy volunteers. RTT cells showed increased levels of nuclear p65 and ASC proteins, pro-IL-1β mRNA, and NLRP3/ASC interaction in basal condition, without any further response upon the LPS + ATP stimuli. Moreover, augmented levels of circulating ASC and IL-18 proteins were found in serum of RTT patients, which are likely able to amplify the inflammatory response. Taken together, our findings suggest that RTT patients exhibited a challenged inflammasome machinery at cellular and systemic level, which may contribute to the subclinical inflammatory state feedback observed in this pathology.
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