Mutations in Hydin impair ciliary motility in mice
Department of Cell Biology; Department of Physiology
Animals; Cell Movement; Cerebral Ventricles; Cerebrospinal Fluid; Cilia; Ependyma; Fluorescent Antibody Technique; Gene Expression Regulation, Developmental; Genetic Predisposition to Disease; Hydrocephalus; Mice; Mice, Knockout; Mice, Transgenic; Microfilament Proteins; Microscopy, Electron, Transmission; Mutation; Respiratory Mucosa; Trachea
Chlamydomonas 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.
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Citation: J Cell Biol. 2008 Feb 11;180(3):633-43. Epub 2008 Feb 4. Link to article on publisher's site
The Journal of cell biology
Lechtreck, Karl-Ferdinand; Delmotte, Philippe; Robinson, Michael L.; Sanderson, Michael J.; and Witman, George B., "Mutations in Hydin impair ciliary motility in mice" (2008). Cell and Developmental Biology Publications and Presentations. Paper 38.