Functional hyperactivity in long QT syndrome type 1 pluripotent stem cell-derived sympathetic neurons
Annika Winbo, Suganeya Ramanan, Emily Eugster, Annika Rydberg, Stefan Jovinge, Jonathan R. Skinner, Johanna M. Montgomery
Abstract
Here, we present the first study of patient-derived LQT1 sympathetic neurons that are norepinephrine secreting, and electrophysiologically functional, in vitro. Our data reveal a novel LQT1 sympathetic neuronal phenotype of increased neurotransmission and excitability. The identified sympathetic neuronal hyperactivity phenotype is of particular relevance as it could contribute to the mechanisms underlying sympathetically triggered arrhythmia in LQT1.
Topics & Concepts
NeuroscienceInduced pluripotent stem cellPhenotypeLong QT syndromeNorepinephrineBiologySympathetic nervous systemMedicineQT intervalDopamineInternal medicineEndocrinologyEmbryonic stem cellGeneticsBlood pressureGeneCardiac electrophysiology and arrhythmiasReceptor Mechanisms and SignalingIon channel regulation and function