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Interregulation between fragile X mental retardation protein and methyl CpG binding protein 2 in the mouse posterior cerebral cortex by Jason Arsenault; Alexander W M Hooper; Shervin Gholizadeh; Tian Kong; Laura K Pacey; Enea Koxhioni; Yosuke Niibori; James H Eubanks; Lu-Yang Wang; David R Hampson is a Biochemistry, Genetics and Molecular Biology article available to read on EtoBox.

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## Abstract Several X-linked neurodevelopmental disorders including Rett syndrome, induced by mutations in the __MECP2__ gene, and fragile X syndrome (FXS), caused by mutations in the __FMR1__ gene, share autism-related features. The mRNA coding for methyl CpG binding protein 2 (MeCP2) has previously been identified as a substrate for the mRNA-binding protein, fragile X mental retardation protein (FMRP), which is silenced in FXS. Here, we report a homeostatic relationship between these two key regulators of gene expression in mouse models of FXS (__Fmr1__ Knockout (KO)) and Rett syndrome (__MeCP2__ KO). We found that the level of MeCP2 protein in the cerebral cortex was elevated in __Fmr1__ KO mice, whereas __MeCP2__ KO mice displayed reduced levels of FMRP, implicating interplay between the activities of MeCP2 and FMRP. Indeed, knockdown of MeCP2 with short hairpin RNAs led to a reduction of FMRP in mouse Neuro2A and in human HEK-293 cells, suggesting a reciprocal coupling in the expression level of these two regulatory proteins. Intra-cerebroventricular injection of an adeno-associated viral vector coding for FMRP led to a concomitant reduction in MeCP2 expression __in vivo__ and

Who reads Interregulation between fragile X mental retardation protein and methyl CpG binding protein 2 in the mouse posterior cerebral cortex?

Author

Jason Arsenault; Alexander W M Hooper; Shervin Gholizadeh; Tian Kong; Laura K Pacey; Enea Koxhioni; Yosuke Niibori; James H Eubanks; Lu-Yang Wang; David R Hampson

Publisher

Oxford University Press (OUP)

Published

2020

Language

EN

Field

Biochemistry, Genetics and Molecular Biology (Life Sciences)