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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.

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An increase in the density of erythrocytes was observed after storage of whole blood for 30 days at 4 degrees C in either acid citrate-dextrose or citrate-phosphate-dextrose-adrenaline. Glucose-6-phosphate dehydrogenase activity in unfractionated red blood cell lysates did not vary with the storage time. Enzyme activity in the lighter fraction separated by density gradient centrifugation was higher than that in heavier fractions. The decline in glucose-6-phosphate dehydrogenase activity with density was less marked after storage of whole blood for 30 days. It is suggested that density modifications are not related to the ageing of erythrocytes and additional mechanisms may be involved.

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