| Autor: |
Cortelazzo A; Child Neuropsychiatry Unit, University Hospital Azienda Ospedaliera Universitaria Senese (AOUS), Viale M. Bracci 16, 53100 Siena, Italy.; Department of Medical Biotechnologies, University of Siena, Via A. Moro 2, 53100 Siena, Italy.; Clinical Pathology Laboratory Unit, University Hospital AOUS, Viale M. Bracci 16, 53100 Siena, Italy., De Felice C; Neonatal Intensive Care Unit, University Hospital AOUS, Viale M. Bracci 16, 53100 Siena, Italy., De Filippis B; Centre for Behavioural Sciences and Mental Health, Istituto Superiore di Sanità (ISS), Viale Regina Elena 299, 00161 Rome, Italy., Ricceri L; Centre for Behavioural Sciences and Mental Health, Istituto Superiore di Sanità (ISS), Viale Regina Elena 299, 00161 Rome, Italy., Laviola G; Centre for Behavioural Sciences and Mental Health, Istituto Superiore di Sanità (ISS), Viale Regina Elena 299, 00161 Rome, Italy., Leoncini S; Child Neuropsychiatry Unit, University Hospital Azienda Ospedaliera Universitaria Senese (AOUS), Viale M. Bracci 16, 53100 Siena, Italy.; Department of Molecular and Developmental Medicine, University of Siena, Via A. Moro 6, 53100 Siena, Italy., Signorini C; Department of Molecular and Developmental Medicine, University of Siena, Via A. Moro 6, 53100 Siena, Italy., Pescaglini M; Clinical Pathology Laboratory Unit, University Hospital AOUS, Viale M. Bracci 16, 53100 Siena, Italy., Guerranti R; Department of Medical Biotechnologies, University of Siena, Via A. Moro 2, 53100 Siena, Italy.; Clinical Pathology Laboratory Unit, University Hospital AOUS, Viale M. Bracci 16, 53100 Siena, Italy., Timperio AM; Department of Ecological and Biological Sciences, University of Tuscia, Largo dell'Università, snc, 01100 Viterbo, Italy., Zolla L; Department of Ecological and Biological Sciences, University of Tuscia, Largo dell'Università, snc, 01100 Viterbo, Italy., Ciccoli L; Department of Molecular and Developmental Medicine, University of Siena, Via A. Moro 6, 53100 Siena, Italy., Hayek J; Child Neuropsychiatry Unit, University Hospital Azienda Ospedaliera Universitaria Senese (AOUS), Viale M. Bracci 16, 53100 Siena, Italy. |
| Abstrakt: |
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. |
