Research ArticleImmunology

Overexpression of Methyl-CpG Binding Protein 2 Impairs TH1 Responses

Science Translational Medicine  05 Dec 2012:
Vol. 4, Issue 163, pp. 163ra158
DOI: 10.1126/scitranslmed.3004430

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The Immune Response in a Bind

Children with MeCP2 (methyl-CpG binding protein 2) duplication syndrome have severe neurological defects caused by the overexpression of the MECP2 gene, which modulates expression of genes that influence neuronal and brain function. Many of these children also have recurrent respiratory infections; however, the role of MECP2 duplication in the immune system has been unclear. Now, Yang et al. have found that MeCP2 duplication may affect T helper type 1 (TH1) immunity.

The authors examined patients with MECP2 duplication and found immunological abnormalities, including differences in memory T and B cells and NK cells. Indeed, mice that overexpress MeCP2 had an immunological defect as well. The immune system has evolved specialized functions to ward off particular types of infections. These MeCP2-overexpressing mice could not mount a sufficient TH1 response to control an infection by the parasite Leishmania major but could fight off an airway fungal infection—a TH2 response. Immune cells from children with MeCP2 duplication syndrome also had defects in the interferon-γ–mediated TH1 response. Yang et al. show that this defect may be caused by MeCP2-suppressing expression of IFN-γ in TH1 cells. This study paves the way for preventing infectious complications in children with MeCP2 duplication syndrome.