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Epac-mediated cAMP-signalling in the mouse model of Rett Syndrome.

S L Mironov1, E Y Skorova, S Kügler

  • 1DFG-Center of Molecular Physiology of the Brain, Germany. smirono@gwdg.de

Neuropharmacology
|January 15, 2011
PubMed
Summary
This summary is machine-generated.

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Rett Syndrome (RTT) is linked to abnormal cAMP levels in the brain, impacting neuronal development and respiratory control. Restoring cAMP levels with PDE4 inhibition improved neuronal function in a mouse model.

Area of Science:

  • Neuroscience
  • Developmental Biology
  • Molecular Biology

Background:

  • Rett Syndrome (RTT) is a neurodevelopmental disorder associated with synaptogenesis and neuronal circuitry deficits.
  • Cyclic adenosine monophosphate (cAMP) is crucial for neuronal outgrowth, plasticity, and regeneration.

Purpose of the Study:

  • To investigate cAMP homeostasis in the pre-Bötzinger complex during early postnatal development in a mouse model of Rett Syndrome (MeCP2-/y mice).
  • To explore the role of cAMP signaling, specifically Epac, in the pathophysiology of RTT and its impact on respiratory circuits.

Main Methods:

  • Quantified cAMP levels and fluctuations in neurons of MeCP2-/y mice using a targeted Epac1-camps sensor.
  • Utilized pharmacological agents like rolipram (PDE4 inhibitor) and 8-pCPT (Epac activator) to modulate cAMP levels and signaling.

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  • Assessed neuronal process elongation and neuronal bursting activity in response to cAMP modulation.

    • Main Results:

    • MeCP2-/y mice exhibited lower resting cAMP levels and reduced amplitude/faster time-course of cAMP transients compared to wild-type.
    • Inhibition of phosphodiesterase PDE4 normalized cAMP levels and transients in mutant mice.
    • Elevated cAMP levels, particularly via Epac activation, promoted neuronal outgrowth and normalized bursting activity in pre-Bötzinger complex neurons of RTT models.

    Conclusions:

    • Disturbances in cAMP homeostasis in RTT mouse models lead to inadequate Epac signaling.
    • Defective cAMP-Epac signaling contributes to the aberrant development of respiratory circuits, potentially causing irregular breathing in RTT.
    • Targeting cAMP pathways, especially Epac, may offer therapeutic strategies for RTT-associated respiratory dysfunction.