β-Catenin Deletion in Regional Neural Progenitors Leads to Congenital Hydrocephalus in Mice.

ABSTRACT: Congenital hydrocephalus is a major neurological disorder with high rates of morbidity and mortality; however, the underlying cellular and molecular mechanisms remain largely unknown. Reproducible animal models mirroring both embryonic and postnatal hydrocephalus are also limited. Here, we describe a new mouse model of congenital hydrocephalus through knockout of β-catenin in Nkx2.1-expressing regional neural progenitors. Progressive ventriculomegaly and an enlarged brain were consistently observed in knockout mice from embryonic day 12.5 through to adulthood. Transcriptome profiling revealed severe dysfunctions in progenitor maintenance in the ventricular zone and therefore in cilium biogenesis after β-catenin knockout. Histological analyses also revealed an aberrant neuronal layout in bo

SUBMITTER: Ma L 

PROVIDER: S-EPMC8782971 | biostudies-literature | 2022 Jan

REPOSITORIES: biostudies-literature