structural defects in cilia of the choroid plexus, subfornical organ and ventricular ependyma are associated with ventriculomegaly结构性缺陷在脉络丛的纤毛,subfornical器官和脑室室管膜与ventriculomegaly相关联.pdfVIP

Swiderski et al. Fluids and Barriers of the CNS 2012, 9:22 FLUIDS AND BARRIERS /content/9/1/22 OF THE CNS RESEARCH Open Access Structural defects in cilia of the choroid plexus, subfornical organ and ventricular ependyma are associated with ventriculomegaly 1 2 3 1 2 2* Ruth E Swiderski , Khristofor Agassandian , Jean L Ross , Kevin Bugge , Martin D Cassell and Charles Yeaman Abstract Background: Hydrocephalus is a heterogeneous disorder with multiple etiologies that are not yet fully understood. Animal models have implicated dysfunctional cilia of the ependyma and choroid plexus in the development of the disorder. In this report, we sought to determine the origin of the ventriculomegaly in four Bardet Biedl syndrome (BBS) mutant mouse strains as models of a ciliopathy. Methods: Evans Blue dye was injected into the lateral ventricle of wild- type and BBS mutant mice to determine whether obstruction of intra- or extra-ventricular CSF flow contributed to ventriculomegaly. Transmission electron microscopy (TEM) was used to examine the ultrastructure of the choroid plexus, subfornical organ (SFO), subcommisural organ (SCO), and ventricular ependyma to evaluate their ultrastructure and the morphology of their primary and motile cilia. Results and discussion: No obstruction of intra- or extra-ventricular CSF flow was observed, implying a communicating form of hydrocephalus in BBS mutant mice. TEM analyses of the mutants showed no evidence of choroidal