... in nonobstructive forms of hydrocephalus. The loss or deformation of cilia on the surface of ChP epithelial cells may alter the function of proteins involved in ion transport and CSF production, similar to what occurs in the renal epithelia of cystic kidney diseases ( Liu et al., 2005 ). We have preliminary...

... the epidermal mucociliary epithelium was reduced. Likewise, Cfap206 knockout mice revealed ciliary phenotypes. Electron tomography of immotile knockout mouse sperm flagella indicated a role in radial spoke formation reminiscent of FAP206 function in Tetrahymena . Male infertility, hydrocephalus and impaired...

...Zakia Abdelhamed; Shawn M. Vuong; Lauren Hill; Crystal Shula; Andrew Timms; David Beier; Kenneth Campbell; Francesco T. Mangano; Rolf W. Stottmann; June Goto ABSTRACT Pediatric hydrocephalus is characterized by an abnormal accumulation of cerebrospinal fluid (CSF) and is one of the most common...

... to dilatation of the brain ventricles during late embryonic stages (ventriculomegaly), which initiates hydrocephalus during postnatal stages. These phenotypes can all be significantly rescued by treatment with the mTORC1 inhibitor rapamycin. These results suggest that primary cilia regulate ventricle...

... that the potassium channels expressed in neuroepithelial cells determine the formation of the ventricular system. The phenotype of a novel zebrafish mutant characterized by denudation of neuroepithelial lining of the ventricular system and hydrocephalus is mechanistically linked to Kcng4b, a homologue of the ‘silent...

..., and hydrocephalus. Here, we explore the connection between cilia dysfunction and the development of hydrocephalus by using the Tg737 orpk mutants. Our analysis indicates that cilia on cells of the brain ventricles of Tg737 orpk mutant mice are severely malformed. On the ependymal cells, these defects lead...

...Albrecht G. Kramer-Zucker; Felix Olale; Courtney J. Haycraft; Bradley K. Yoder; Alexander F. Schier; Iain A. Drummond Cilia, as motile and sensory organelles, have been implicated in normal development, as well as diseases including cystic kidney disease,hydrocephalus and situs inversus. In kidney...

...Perry J. Blackshear; Joan P. Graves; Deborah J. Stumpo; Inma Cobos; John L. R. Rubenstein; Darryl C. Zeldin One line of mice harboring a cardiac-specific epoxygenase transgene developed head swelling and rapid neurological decline in young adulthood, and had marked hydrocephalus of the lateral...

... suggests that subcommissural organ failure is the main cause of prenatal hydrocephalus observed in both strains. The formation of the posterior commissure is also delayed, and errors in axonal pathfinding are frequent. In chick, ectopic expression of En by in ovo electroporation, affects growth...