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Altered erythrocyte membrane fatty acid profile in typical Rett syndrome: effects of omega-3 polyunsaturated fatty acid supplementation.

Abstract
This study mainly aims at examining the erythrocyte membrane fatty acid (FAs) profile in Rett syndrome (RTT), a genetically determined neurodevelopmental disease. Early reports suggest a beneficial effects of omega-3 polyunsaturated fatty acids (ω-3 PUFAs) on disease severity in RTT. A total of 24 RTT patients were assigned to ω-3 PUFAs-containing fish oil for 12 months in a randomized controlled study (average DHA and EPA doses of 72.9, and 117.1mg/kgb.w./day, respectively). A distinctly altered FAs profile was detectable in RTT, with deficient ω-6 PUFAs, increased saturated FAs and reduced trans 20:4 FAs. FAs changes were found to be related to redox imbalance, subclinical inflammation, and decreased bone density. Supplementation with ω-3 PUFAs led to improved ω-6/ω-3 ratio and serum plasma lipid profile, decreased PUFAs peroxidation end-products, normalization of biochemical markers of inflammation, and reduction of bone hypodensity as compared to the untreated RTT group. Our data indicate that a significant FAs abnormality is detectable in the RTT erythrocyte membranes and is partially rescued by ω-3 PUFAs.
AuthorsCinzia Signorini, Claudio De Felice, Silvia Leoncini, Thierry Durand, Jean-Marie Galano, Alessio Cortelazzo, Gloria Zollo, Roberto Guerranti, Stefano Gonnelli, Carla Caffarelli, Marcello Rossi, Alessandra Pecorelli, Giuseppe Valacchi, Lucia Ciccoli, Joussef Hayek
JournalProstaglandins, leukotrienes, and essential fatty acids (Prostaglandins Leukot Essent Fatty Acids) Vol. 91 Issue 5 Pg. 183-93 (Nov 2014) ISSN: 1532-2823 [Electronic] Scotland
PMID25240461 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
CopyrightCopyright © 2014 Elsevier Ltd. All rights reserved.
Chemical References
  • Biomarkers
  • Fatty Acids, Omega-3
  • Isoprostanes
  • Lipids
  • Docosahexaenoic Acids
  • Eicosapentaenoic Acid
Topics
  • Adolescent
  • Adult
  • Animals
  • Biomarkers (blood)
  • Child
  • Child, Preschool
  • Dietary Supplements
  • Docosahexaenoic Acids (administration & dosage, metabolism)
  • Eicosapentaenoic Acid (administration & dosage, metabolism)
  • Erythrocyte Membrane (drug effects, metabolism, pathology)
  • Fatty Acids, Omega-3 (administration & dosage, metabolism)
  • Female
  • Humans
  • Inflammation (metabolism, pathology)
  • Isoprostanes (blood)
  • Lipids (blood)
  • Rett Syndrome (diet therapy, metabolism, pathology)

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