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

Joint Authors

Laviola, Giovanni
Guerranti, Roberto
De Filippis, Bianca
Ricceri, Laura
Pescaglini, Monica
Timperio, Anna Maria
Zolla, Lello
Hayek, Joussef
Leoncini, Silvia
Cortelazzo, Alessio
Signorini, Cinzia
Ciccoli, Lucia
De Felice, Claudio

Source

Mediators of Inflammation

Issue

Vol. 2017, Issue 2017 (31 Dec. 2017), pp.1-9, 9 p.

Publisher

Hindawi Publishing Corporation

Publication Date

2017-05-16

Country of Publication

Egypt

No. of Pages

9

Main Subjects

Diseases

Abstract EN

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.

American Psychological Association (APA)

Cortelazzo, Alessio& De Felice, Claudio& De Filippis, Bianca& Ricceri, Laura& Laviola, Giovanni& Leoncini, Silvia…[et al.]. 2017. Persistent Unresolved Inflammation in the Mecp2-308 Female Mutated Mouse Model of Rett Syndrome. Mediators of Inflammation،Vol. 2017, no. 2017, pp.1-9.
https://search.emarefa.net/detail/BIM-1188825

Modern Language Association (MLA)

Cortelazzo, Alessio…[et al.]. Persistent Unresolved Inflammation in the Mecp2-308 Female Mutated Mouse Model of Rett Syndrome. Mediators of Inflammation No. 2017 (2017), pp.1-9.
https://search.emarefa.net/detail/BIM-1188825

American Medical Association (AMA)

Cortelazzo, Alessio& De Felice, Claudio& De Filippis, Bianca& Ricceri, Laura& Laviola, Giovanni& Leoncini, Silvia…[et al.]. Persistent Unresolved Inflammation in the Mecp2-308 Female Mutated Mouse Model of Rett Syndrome. Mediators of Inflammation. 2017. Vol. 2017, no. 2017, pp.1-9.
https://search.emarefa.net/detail/BIM-1188825

Data Type

Journal Articles

Language

English

Notes

Includes bibliographical references

Record ID

BIM-1188825