%0 Journal Article %J The Journal of Cell Biology %D 2008 %T Mutations in Hydin impair ciliary motility in mice %A Lechtreck, Karl-Ferdinand %A Delmotte, P. %A Robinson, M. L. %A Sanderson, M. J. %A Witman, G. B. %X

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.

%B The Journal of Cell Biology %V 180 %P 633 - 643 %8 02/2008 %@ 0021-95251540-8140 %G eng %U http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2234243/ %N 3 %1

200710162[PII]18250199[pmid]18250199[pmid]

%! J Cell Biol