Cognitive impairment in people with epilepsy

A Novak, K Vizjak, M Rakusa - Journal of Clinical Medicine, 2022 - mdpi.com
People with epilepsy frequently have cognitive impairment. The majority of cognitive
problems is influenced by a variety of interlinked factors, including the early onset of …

Developmental and epileptic encephalopathies

IE Scheffer, S Zuberi, HC Mefford, R Guerrini… - Nature Reviews …, 2024 - nature.com
Developmental and epileptic encephalopathies, the most severe group of epilepsies, are
characterized by seizures and frequent epileptiform activity associated with developmental …

Reduced synaptic activity and dysregulated extracellular matrix pathways in midbrain neurons from Parkinson's disease patients

S Stern, S Lau, A Manole, I Rosh, MM Percia… - npj Parkinson's …, 2022 - nature.com
Several mutations that cause Parkinson's disease (PD) have been identified over the past
decade. These account for 15–25% of PD cases; the rest of the cases are considered …

IQSEC2 mutation associated with epilepsy, intellectual disability, and autism results in hyperexcitability of patient-derived neurons and deficient synaptic transmission

B Brant, T Stern, HA Shekhidem, L Mizrahi, I Rosh… - Molecular …, 2021 - nature.com
Mutations in the IQSEC2 gene are associated with drug-resistant, multifocal infantile and
childhood epilepsy; autism; and severe intellectual disability (ID). We used induced …

Severe deficiency of the voltage-gated sodium channel NaV1. 2 elevates neuronal excitability in adult mice

J Zhang, X Chen, M Eaton, J Wu, Z Ma, S Lai, A Park… - Cell reports, 2021 - cell.com
Scn2a encodes the voltage-gated sodium channel Na V 1.2, a main mediator of neuronal
action potential firing. The current paradigm suggests that Na V 1.2 gain-of-function variants …

Hyperexcitability and pharmacological responsiveness of cortical neurons derived from human iPSCs carrying epilepsy-associated sodium channel Nav1. 2-L1342P …

Z Que, MI Olivero-Acosta, J Zhang, M Eaton… - Journal of …, 2021 - Soc Neuroscience
With the wide adoption of genomic sequencing in children having seizures, an increasing
number of SCN2A genetic variants have been revealed as genetic causes of epilepsy …

Potassium channelopathies associated with epilepsy-related syndromes and directions for therapeutic intervention

VK Gribkoff, RJ Winquist - Biochemical Pharmacology, 2023 - Elsevier
A number of mutations to members of several CNS potassium (K) channel families have
been identified which result in rare forms of neonatal onset epilepsy, or syndromes of which …

Multielectrode arrays for functional phenoty** of neurons from induced pluripotent stem cell models of neurodevelopmental disorders

FP McCready, S Gordillo-Sampedro, K Pradeepan… - Biology, 2022 - mdpi.com
Simple Summary Multielectrode array technology allows researchers to record the
spontaneous firing activity of cultured neurons over a period of multiple weeks or months …

Genome editing in iPSC-based neural systems: from disease models to future therapeutic strategies

A McTague, G Rossignoli, A Ferrini, S Barral… - Frontiers in Genome …, 2021 - frontiersin.org
Therapeutic advances for neurological disorders are challenging due to limited accessibility
of the human central nervous system and incomplete understanding of disease …

Early maturation and hyperexcitability is a shared phenotype of cortical neurons derived from different ASD-associated mutations

Y Hussein, U Tripathi, A Choudhary, R Nayak… - Translational …, 2023 - nature.com
Abstract Autism Spectrum Disorder (ASD) is characterized mainly by social and sensory-
motor abnormal and repetitive behavior patterns. Over hundreds of genes and thousands of …