Mutations in Hydin impair ciliary motility in mice

Bibliographic Collection: 
MOCA Reference, APE
Publication Type: Journal Article
Authors: Lechtreck, Karl-Ferdinand; Delmotte, P.; Robinson, M. L.; Sanderson, M. J.; Witman, G. B.
Year of Publication: 2008
Journal: The Journal of Cell Biology
Volume: 180
Issue: 3
Pagination: 633 - 643
Date Published: 02/2008
Publication Language: eng
ISBN Number: 0021-95251540-8140

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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Short Title: J Cell Biol