Research demonstrates altered glycosylation and impaired neuronal maturation in iPSC-derived cortical organoids, suggesting impacts on brain network activity.
Key Points
ALG13-CDG models show significant glycosylation defects affecting critical proteins for neuronal function.
Multiomics analysis revealed dysregulation in pathways linked to neuronal development and excitatory/inhibitory balance.
Electrophysiological recordings indicated network hypoactivity with reduced neuron firing rates and immature dynamics.
The findings provide insights into potential therapeutic targets for CDG and related disorders with similar neurological traits.