Lechtreck, Karl-FerdinandDelmotte, PhilippeRobinson, Michael L.Sanderson, Michael J.Witman, George B.2022-08-232022-08-232008-02-062008-12-11J Cell Biol. 2008 Feb 11;180(3):633-43. Epub 2008 Feb 4. <a href="http://dx.doi.org/10.1083/jcb.200710162">Link to article on publisher's site</a>1540-8140 (Electronic)10.1083/jcb.20071016218250199https://hdl.handle.net/20.500.14038/26536Chlamydomonas reinhardtii hydin is a central pair protein required for flagellar motility, and mice with Hydin defects develop lethal hydrocephalus. To determine if defects in Hydin cause hydrocephalus through a mechanism involving cilia, we compared the morphology, ultrastructure, and activity of cilia in wild-type and hydin mutant mice strains. The length and density of cilia in the brains of mutant animals is normal. The ciliary axoneme is normal with respect to the 9 + 2 microtubules, dynein arms, and radial spokes but one of the two central microtubules lacks a specific projection. The hydin mutant cilia are unable to bend normally, ciliary beat frequency is reduced, and the cilia tend to stall. As a result, these cilia are incapable of generating fluid flow. Similar defects are observed for cilia in trachea. We conclude that hydrocephalus in hydin mutants is caused by a central pair defect impairing ciliary motility and fluid transport in the brain.en-USAnimalsCell MovementCerebral VentriclesCerebrospinal FluidCiliaEpendymaFluorescent Antibody TechniqueGene Expression Regulation, DevelopmentalGenetic Predisposition to DiseaseHydrocephalusMiceMice, KnockoutMice, TransgenicMicrofilament ProteinsMicroscopy, Electron, TransmissionMutationRespiratory MucosaTracheaCell BiologyJournal Articlehttps://escholarship.umassmed.edu/cellbiology_pp/38680180cellbiology_pp/38