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Altered neuronal network and rescue in a human MECP2 duplication model.

S Nageshappa1, C Carromeu2,3, C A Trujillo2,3

  • 1Department of Human Genetics, Laboratory for the Genetics of Cognition, Center for Human Genetics, KU Leuven, Leuven, Belgium.

Molecular Psychiatry
|September 9, 2015
PubMed
Summary

Increased dosage of methyl-CpG-binding protein-2 (MeCP2) causes severe neurodevelopmental issues. Researchers used patient stem cells to find a potential drug, NCH-51, that may reverse these effects in MECP2 duplication syndrome.

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Area of Science:

  • Neuroscience
  • Genetics
  • Stem Cell Biology

Background:

  • Overexpression of methyl-CpG-binding protein-2 (MeCP2) causes severe neurodevelopmental disorders.
  • The MECP2 duplication syndrome (MECP2dup) is characterized by increased MeCP2 dosage and presents at birth.

Purpose of the Study:

  • To investigate the impact of elevated MeCP2 levels on human neurons using patient-derived induced pluripotent stem cells (iPSCs).
  • To identify potential therapeutic interventions for MECP2dup by screening epigenetic compounds.

Main Methods:

  • Generation of iPSCs from MECP2dup patients with varying duplication sizes.
  • Differentiation of iPSCs into cortical neurons for functional analysis.
  • Assessment of synaptogenesis, dendritic complexity, and neuronal network synchronization using multi-electrode arrays.
  • Screening of epigenetic compounds for therapeutic potential.

Main Results:

  • MECP2dup-derived neurons exhibited increased synaptogenesis and dendritic complexity.
  • Neuronal network synchronization was altered in MECP2dup neurons.
  • The histone deacetylase inhibitor NCH-51 demonstrated potential to reverse MECP2dup-associated alterations.

Conclusions:

  • Patient-derived iPSC models accurately recapitulate early MECP2dup phenotypes.
  • NCH-51 represents a novel therapeutic candidate for MECP2dup.
  • This cellular model provides a platform for drug discovery in severe neurodevelopmental disorders.