Amplification of human interneuron progenitors promotes brain tumors and neurological defects.

ABSTRACT: Evolutionary development of the human brain is characterized by the expansion of various brain regions. Here, we show that developmental processes specific to humans are responsible for malformations of cortical development (MCDs), which result in developmental delay and epilepsy in children. We generated a human cerebral organoid model for tuberous sclerosis complex (TSC) and identified a specific neural stem cell type, caudal late interneuron progenitor (CLIP) cells. In TSC, CLIP cells over-proliferate, generating excessive interneurons, brain tumors, and cortical malformations. Epidermal growth factor receptor inhibition reduces tumor burden, identifying potential treatment options for TSC and related disorders. The identification of CLIP cells reveals the extended interneuron generatio

SUBMITTER: Eichmuller OL 

PROVIDER: S-EPMC7613689 | biostudies-literature | 2022 Jan

REPOSITORIES: biostudies-literature