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Mediators of Inflammation
Volume 2017, Article ID 9467819, 9 pages
Research Article

Persistent Unresolved Inflammation in the Mecp2-308 Female Mutated Mouse Model of Rett Syndrome

1Child Neuropsychiatry Unit, University Hospital Azienda Ospedaliera Universitaria Senese (AOUS), Viale M. Bracci 16, 53100 Siena, Italy
2Department of Medical Biotechnologies, University of Siena, Via A. Moro 2, 53100 Siena, Italy
3Clinical Pathology Laboratory Unit, University Hospital AOUS, Viale M. Bracci 16, 53100 Siena, Italy
4Neonatal Intensive Care Unit, University Hospital AOUS, Viale M. Bracci 16, 53100 Siena, Italy
5Centre for Behavioural Sciences and Mental Health, Istituto Superiore di Sanità (ISS), Viale Regina Elena 299, 00161 Rome, Italy
6Department of Molecular and Developmental Medicine, University of Siena, Via A. Moro 6, 53100 Siena, Italy
7Department of Ecological and Biological Sciences, University of Tuscia, Largo dell’Università, snc, 01100 Viterbo, Italy

Correspondence should be addressed to Alessio Cortelazzo; moc.liamg@elaetroc

Received 6 December 2016; Accepted 19 April 2017; Published 16 May 2017

Academic Editor: Chiara De Luca

Copyright © 2017 Alessio Cortelazzo et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Rett syndrome (RTT) is a rare neurodevelopmental disorder usually caused by mutations in the X-linked gene methyl-CpG-binding protein 2 (MECP2). Several Mecp2 mutant mouse lines have been developed recapitulating part of the clinical features. In particular, Mecp2-308 female heterozygous mice, bearing a truncating mutation, are a validated model of the disease. While recent data suggest a role for inflammation in RTT, little information on the inflammatory status in murine models of the disease is available. Here, we investigated the inflammatory status by proteomic 2-DE/MALDI-ToF/ToF analyses in symptomatic Mecp2-308 female mice. Ten differentially expressed proteins were evidenced in the Mecp2-308 mutated plasma proteome. In particular, 5 positive acute-phase response (APR) proteins increased (i.e., kininogen-1, alpha-fetoprotein, mannose-binding protein C, alpha-1-antitrypsin, and alpha-2-macroglobulin), and 3 negative APR reactants were decreased (i.e., serotransferrin, albumin, and apolipoprotein A1). CD5 antigen-like and vitamin D-binding protein, two proteins strictly related to inflammation, were also changed. These results indicate for the first time a persistent unresolved inflammation of unknown origin in the Mecp2-308 mouse model.