The microcephaly gene Donson is essential for progenitors of cortical glutamatergic and GABAergic neurons.

ABSTRACT: Biallelic mutations in DONSON, an essential gene encoding for a replication fork protection factor, were linked to skeletal abnormalities and microcephaly. To better understand DONSON function in corticogenesis, we characterized Donson expression and consequences of conditional Donson deletion in the mouse telencephalon. Donson was widely expressed in the proliferation and differentiation zones of the embryonic dorsal and ventral telencephalon, which was followed by a postnatal expression decrease. Emx1-Cre-mediated Donson deletion in progenitors of cortical glutamatergic neurons caused extensive apoptosis in the early dorsomedial neuroepithelium, thus preventing formation of the neocortex and hippocampus. At the place of the missing lateral neocortex, these mutants exhibited a dorsal exte

SUBMITTER: Venkataramanappa S 

PROVIDER: S-EPMC8011756 | biostudies-literature | 2021 Mar

REPOSITORIES: biostudies-literature