Cytokine Dysregulation in MECP2- and CDKL5-Related Rett Syndrome: Relationships with Aberrant Redox Homeostasis, Inflammation, and ω-3 PUFAs
Open accessAbstract
An involvement of the immune system has been suggested in Rett syndrome (RTT), a devastating neurodevelopmental disorder related to oxidative stress, and caused by a mutation in the methyl-CpG binding protein 2 gene (MECP2) or, more rarely, cyclin-dependent kinase-like 5 (CDKL5). To date, it is unclear whether both mutations may have an impact on the circulating cytokine patterns. In the present study, cytokines involved in the Th1-, Th2-, and T regulatory (T-reg) response, as well as chemokines, were investigated in MECP2- (MECP2-RTT) (n=16) and CDKL5-Rett syndrome (CDKL5-RTT) (n=8), before and after ω-3 polyunsaturated fatty acids (PUFAs) supplementation. A major cytokine dysregulation was evidenced in untreated RTT patients. In MECP2-RTT, a Th2-shifted balance was evidenced, whereas in CDKL5-RTT both Th1- and Th2-related cytokines (except for IL-4) were upregulated. In MECP2-RTT, decreased levels of IL-22 were observed, whereas increased IL-22 and T-reg cytokine levels were evidenced in CDKL5-RTT. Chemokines were unchanged. The cytokine dysregulation was proportional to clinical severity, inflammatory status, and redox imbalance. Omega-3 PUFAs partially counterbalanced cytokine changes, as well as aberrant redox homeostasis and the inflammatory status. RTT is associated with a subclinical immune dysregulation as the likely consequence of a defective inflammation regulatory signaling system.
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Inflammation is a component of pathology in both typical and atypical cases of RTT.
“Additionally, abnormal lipid metabolism is directly linked to inflammation [204], which is a component of pathology in both typical and atypical cases of RTT [205].”
From Rett syndrome: a neurological disorder with metabolic components (2018) · cites this paper as [205]
The Discussion reports a significant proinflammatory status in typical MECP2-RTT. It also reports cytokine dysregulation in CDKL5-RTT, but says this did not increase inflammatory marker levels and that its inflammatory status remains unclear. The claim therefore overstates the evidence for atypical CDKL5-RTT. (LLM, conf=0.88)
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- Authors
- Leoncini, S., De Felice, C., Signorini, C., Zollo, G., Cortelazzo, A., Durand, T., Galano, J.-M., Guerranti, R., Rossi, M., Ciccoli, L., & Hayek, J.
- Affiliations
- University of Siena (IT)
- Neonatal Intensive Care Unit, University Hospital, AOUS, Policlinico “S. M. alle Scotte”, 53100 Siena, Italy
- École Nationale Supérieure de Chimie de Montpellier (FR)
- Respiratory Pathophysiology and Rehabilitation Unit, University Hospital, AOUS, Policlinico “S. M. alle Scotte”, 53100 Siena, Italy
- Ospedale Santa Maria alle Scotte (IT)
- Corresponding author
- Leoncini, Silvia
- ORCID
- Venue
- Oxidative Medicine and Cellular Longevity
- Publisher
- Wiley
- Published
- 2015
- Volume
- 2015
- Pages
- 1-18
- ISSN
- 1942-0994 (ISSN-L)
- 1942-0900
- PMC
- PMC4510261
- OpenAlex
- W2111993591
- Cited by
- 71
- References
- 7
- Citations by year
- 2026: 3 · 2025: 6 · 2024: 9 · 2023: 11 · 2022: 1 · 2021: 7 · 2020: 6 · 2019: 7 · 2018: 7 · 2017: 8 · 2016: 5 · 2015: 1
- FWCI
- 4.81
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Locations
- Oxidative Medicine and Cellular Longevity (publishedVersion, cc-by)
- Europe PMC (PubMed Central) (publishedVersion, cc-by)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- HAL (submittedVersion, cc-by)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion)
- Use Siena air (University of Siena) (submittedVersion, cc-by)
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Genetics › Genetics and Neurodevelopmental Disorders
- Life Sciences › Immunology and Microbiology › Immunology › Immunodeficiency and Autoimmune Disorders
- Life Sciences › Neuroscience › Biological Psychiatry › Tryptophan and brain disorders
- Keywords
- Rett syndrome, MeCP2, CDKL5, cytokine imbalance, omega-3 polyunsaturated fatty acids, redox homeostasis, oxidative stress, inflammation, Th1/Th2 balance, regulatory T cells, IL-22, chemokines, immune dysregulation, neurodevelopmental disorders
- MeSH terms
- Adolescent, Adult, Blood Sedimentation, Child, Child, Preschool, Enzyme-Linked Immunosorbent Assay, Erythrocytes, Female, Humans, Inflammation, Severity of Illness Index, Rett Syndrome, Fatty Acids, Omega-3, Cytokines, Protein Serine-Threonine Kinases, Th1 Cells, Th2 Cells, Dietary Supplements, Polymorphism, Single Nucleotide, T-Lymphocytes, Regulatory, Methyl-CpG-Binding Protein 2, Young Adult
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
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