@article {310573, title = {Mutations in Hydin impair ciliary motility in mice}, journal = {The Journal of Cell Biology}, volume = {180}, year = {2008}, month = {02/2008}, pages = {633 - 643}, 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.

}, isbn = {0021-95251540-8140}, url = {http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2234243/}, author = {Lechtreck, Karl-Ferdinand and Delmotte, P. and Robinson, M. L. and Sanderson, M. J. and Witman, G. B.} }