The ciliary kinesin KIF7 controls the development of the cerebral cortex by acting differentially on SHH signaling in dorsal and ventral forebrain.

ABSTRACT: Mutations of KIF7, a key ciliary component of Sonic Hedgehog (SHH) pathway, are associated in humans with cerebral cortex malformations and clinical features suggestive of cortical dysfunction. KIF7 regulates the processing of GLI-A and GLI3-R transcription factors in a SHH-dependent manner both in humans and in mice. Here, we examine the embryonic cortex development of a mouse model that lacks the expression of KIF7 (Kif7^-/-). The cortex is composed of principal neurons generated locally in the dorsal telencephalon where SHH expression is low and inhibitory interneurons (cIN) generated in the ventral telencephalon where SHH expression is high. We observe a strong impact of Kif7 deletion on the dorsal cortex development whose abnormalities resemble those of

SUBMITTER: Pedraza M 

PROVIDER: S-EPMC12440355 | biostudies-literature | 2025 Sep

REPOSITORIES: biostudies-literature