Loss of Rsph9 causes neonatal hydrocephalus with abnormal development of motile cilia in mice.

ABSTRACT: Hydrocephalus is a brain disorder triggered by cerebrospinal fluid accumulation in brain cavities. Even though cerebrospinal fluid flow is known to be driven by the orchestrated beating of the bundled motile cilia of ependymal cells, little is known about the mechanism of ciliary motility. RSPH9 is increasingly becoming recognized as a vital component of radial spokes in ciliary "9 + 2" ultrastructure organization. Here, we show that deletion of the Rsph9 gene leads to the development of hydrocephalus in the early postnatal period. However, the neurodevelopment and astrocyte development are normal in embryonic Rsph9^-/- mice. The tubular structure of the central aqueduct was comparable in Rsph9^-/- mice. Using high-speed video microscopy, we visualized lower beating amp

SUBMITTER: Zou W 

PROVIDER: S-EPMC7382491 | biostudies-literature | 2020 Jul

REPOSITORIES: biostudies-literature