Topic: human astrocytes

Koolen-de Vries syndrome (KdVS) is a rare genetic disorder marked by intellectual disability and epilepsy, resulting from haploinsufficiency of KANSL1. This gene encodes a scaffolding protein within the non-specific lethal (NSL) complex which is crucial for histone modifications. The NSL complex regulates the expression of housekeeping, DNA repair, and autophagy genes. Autophagy, a pivotal process delivering cytoplasmic material to the lysosome for degradation, plays a key role in brain development and homeostasis maintenance; defects in autophagy have been linked to neurodevelopmental disorde

With the prospect of better translation compared to mouse systems, human IPSC-derived neuronal networks are a promising model system to overcome the valley of death in cognitive drug discovery for neurodegenerative disease. Synapse strengthening may prevent or reverse neurodegeneration. However, screening for compounds that achieve this requires a network capable of synapse strengthening and weakening that is supported by human astrocytes and microglia, i.e. a fully human model system of a minimal brain circuit.So far, convincing post-synaptic immunostains of hIPSC-derived neurons are rare, as