Title

Mutations in Hydin impair ciliary motility in mice

UMMS Affiliation

Department of Cell Biology; Department of Physiology

Date

2-6-2008

Document Type

Article

Subjects

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

Abstract

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.

Rights and Permissions

Citation: J Cell Biol. 2008 Feb 11;180(3):633-43. Epub 2008 Feb 4. Link to article on publisher's site

Related Resources

Link to Article in PubMed

Journal Title

The Journal of cell biology

PubMed ID

18250199