Requirements for the neurodevelopmental disorder-associated gene ZNF292 in human cortical interneuron development and function.

ABSTRACT: Pathogenic mutation of the zinc-finger transcription factor ZNF292 is a recently defined contributor to human neurodevelopmental disorders (NDDs). However, the gene's roles in cortical development and regulatory networks under its control were previously undefined. Here, human stem cell models of ZNF292 deficiency, resembling pathogenic haploinsufficiency, are used to derive cortical inhibitory neuron progenitors and neurons. ZNF292-deficient progenitors undergo precocious differentiation but subsequently exhibit compromised interneuron maturation and function. In progenitors, genome-wide occupancy and transcriptomic analyses identify direct target genes controlling neuronal differentiation and synapse formation that are upregulated upon ZNF292 deficiency. By contrast, deficiency in intern

SUBMITTER: Kaushik K 

PROVIDER: S-EPMC12283078 | biostudies-literature | 2025 May

REPOSITORIES: biostudies-literature