🧠 KCNQ2-related epilepsy as a moving target, not a static phenotype. In a new
@Brain1878 paper (
@OxUniPress), Dirkx et al. follow human iPSC-derived cortical iNeurons carrying KCNQ2 loss-of-function variants across maturation and show that the disease phenotype evolves over time.
At early developmental stages, the hallmark of the disease lies in Kv7-driven hyperexcitability. At later stages, this intrinsic hyperexcitability normalizes, giving way to network phenotypes that diverge further from control trajectories. Together, these data suggest that the primary Kv7.2 defect triggers compensatory responses that become maladaptive as the network matures.
How did they gain all this functional information? By using the MaxTwo High-Density Microelectrode Array (HD-MEA) system. With MaxTwo HD-MEA 6-Well Plate, the team tracked the emergence of spontaneous firing and network bursts as hallmarks of early hyperexcitability, captured the later divergence in network behavior, and showed that retigabine can rescue the early Kv7-driven phenotype but not the later maladaptive remodeling. All of this on one single platform.
👉 Read the full publication here –
mxwbio.com/resources/brain-2…
👏 Congratulations to
@DirkxNina, Dr.
@mark_kaji Kaji, Els De Vriendt, Dr. Giusy Carleo, Dr. Francesco Miceli, Dr. Bob Asselbergh, Dr. Peter Verstraelen, Dr. Noortje Zonnekein, Dr. Lidia Carotenuto, Dr. Louis Dang, Vera Sommers, Eve Vlaemynck, Prof. Dr.
@LievenLagae, Dr. Berten Ceulemans, Prof. Dr. Peter De Jonghe, Prof. Dr. Winnok De Vos, Prof. Dr. Maurizio Taglialatela, and Dr.
@SWeckhuysen from:
@Weckhuysen_team , Translational Epilepsy Genomics Group, VIB-UAntwerp Center for Molecular Neurology (
@CMN_VIB), VIB (
@VIBLifeSciences), Department of Biomedical Sciences, University of Antwerp, Translational Neurosciences, Faculty of Medicine and Health Science, University of Antwerp, µNEURO Research Centre of Excellence, University of Antwerp, Division of Pharmacology, Department of Neuroscience, University of Naples Federico II, Laboratory of Cell Biology and Histology, University of Antwerp, Division of Pediatric Neurology, Department of Pediatrics, Michigan Medicine (
@umichmedicine), University of Michigan (
@UMich), Department of Development and Regeneration, Paediatric Neurology, University of Leuven (
@KU_Leuven), Member of European Reference Network EpiCare, Department of Pediatric Neurology, University Hospital Antwerp, Laboratory of Neuromuscular Pathology, Institute Born-Bunge, University of Antwerp, Neuromuscular Reference Centre, Department of Neurology, Antwerp University Hospital, Antwerp Centre for Advanced Microscopy, University of Antwerp, and Division of Neurology, University Hospital Antwerp
#HDMEA #Electrophysiology #Neuroscience