3. Electrical activity in motoneuron diseases

Chloride channels and cation-chloride cotransporters play in concert to control neuronal excitability. Loss of function of potassium-chloride KCC3 in humans and mice induces severe sensory-motor neuropathy and decreased KCC2 is responsible for spasticity following spinal cord trauma. Expression of calcium-activated chloride channels in spinal motoneurons could play a role in the control of motoneuron excitability under pathophysiological conditions. Our research goal is to uncover cellular mechanisms linking chloride homeostasis to motoneuron excitability in a neuroinflammatory context.

Motoneuron 3.1


RNA interference using single cell electroporation for gene identification of  calcium-activated chloride current recorded in motoneurons (red transfected Hb9::GFP motoneuron). Anoctamins genes are developmentally regulated in motoneurons.

 

 

 

Motoneuron 3.2


Motoneuron electrical activity.

 

 

 

Motoneuron 3.3

We study the contribution of the cation-chloride co-transporters in the pathophysiology of motoneurons by studying KCC3-deficent mice. KCC3 mutant mice develop a severe motor syndrome resembling Anderman syndrome. One motor efferent fiber (green) contact several muscle endplates (red).

 

Major publications

Lucas et al., Mol. Cell. Neurosci, 50: 211-20, 2012
Pieraut et al., J. Neurosci, 31: 13516-26 , 2011
Boudes et al., J. Neurosci, 29: 10063-71, 2009
Pieraut et al., J. Neurosci, 27: 6751-6759, 2007
Dubreuil et al., J. Neurosci, 24: 8480-8484, 2004

Collaborations

  • Eric Delpire (University, USA)
  • Guy Rouleau (Montreal University, Canada)

Contact

Frédérique Scamps