FMRP regulates adult human cortical neuron excitability via cyclic-AMP signalling
Max J J Knops, Soraya MeftahiD, Max A WilsoniD, Lewis W. TayloriD, Calum BonthroniD, Alsadeg BilaliD, Imran Liaquat, Paul Martin BrennaniD, Claire S. DurrantiD, Sam A. BookeriD
Abstract
Fragile X Syndrome (FXS) is a common inherited neurodevelopmental condition, resulting from loss of Fragile X Messenger Ribonuclear Protein (FMRP). Rodent models of FXS display cellular hyperexcitability, but it is not known to what extent this is the case in intact human neurons. Depleting FMRP in human brain slice cultures reveals cyclic-AMP-dependent cellular hyperexcitability which is corrected by phosphodiesterase 4D inhibition and may be independent of neurodevelopment.
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