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KCNQ2/3 Gain-of-Function Variants and Cell Excitability: Differential Effects in CA1 versus L2/3 Pyramidal Neurons.

Nissi Varghese1, Bruno Moscoso2, Ana Chavez2

  • 1Department of Physiology and Neurobiology, University of Connecticut, Storrs, Connecticut 06269.

The Journal of Neuroscience : the Official Journal of the Society for Neuroscience
|August 22, 2023
PubMed
Summary

Gain-of-function variants in KCNQ2/3 potassium channels cause neurodevelopmental disorders. This study reveals these variants can paradoxically increase or decrease neuronal excitability, depending on the neuron type, impacting brain function.

Keywords:
KCNQ2KCNQ3gain-of-functionhippocampusneurological disorderspotassium channels

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Area of Science:

  • Neuroscience
  • Molecular Biology
  • Genetics

Background:

  • Gain-of-function (GOF) variants in KCNQ2 and KCNQ3 potassium channels are linked to epilepsy and autism spectrum disorders.
  • The precise cellular mechanisms by which these variants disrupt forebrain function remain largely unknown.

Purpose of the Study:

  • To investigate the differential effects of KCNQ2 and KCNQ3 gain-of-function variants on neuronal excitability in distinct forebrain pyramidal neuron populations.
  • To elucidate the cellular basis for the diverse neurological phenotypes associated with KCNQ2/3 GOF variants.

Main Methods:

  • Utilized a series of transgenic mouse models expressing specific KCNQ2 (R201C) and KCNQ3 (R231C) gain-of-function variants.
  • Performed electrophysiological recordings on layer 2/3 (L2/3) and CA1 pyramidal neurons in the mouse forebrain.
  • Analyzed neuronal excitability changes in response to KCNQ2/3 GOF variant expression.

Main Results:

  • The KCNQ2 R201C variant induced hyperexcitability in L2/3 pyramidal neurons but hypoexcitability in CA1 pyramidal neurons.
  • The KCNQ3 R231C variant similarly caused hyperexcitability in L2/3 neurons and hypoexcitability in superficial CA1 neurons.
  • Expression of the KCNQ2 GOF variant R201C in forebrain neurons was associated with seizures and sudden unexpected death in epilepsy (SUDEP).

Conclusions:

  • KCNQ2/3 gain-of-function variants exhibit cell-type-specific effects on neuronal excitability within the forebrain.
  • These differential effects on pyramidal neurons contribute to the complexity of neurodevelopmental disorders linked to KCNQ2/3 channelopathies.
  • Findings provide a framework for understanding how potassium channel dysfunction leads to varied neurological phenotypes.