HNRNPU and developmental and epileptic encephalopathy, 54: Here, we model microdeletions of the HNRNPU locus responsible of HNRNPU-related disorder and provide novel evidence of the molecular and cellular consequences of HNRNPU deficiency in differentiating human neuronal cells with hindbrain phenotype, adding knowledge on the effect HNRNPU deficiency on the brain region where HNRNPU expression is the highest.