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When cilia lose the beat

Normal cilia beat smoothly (top), but hydin-lacking cilia (bottom) are stiff.

Like a rower who stops midstroke, cilia lacking a particular protein slack off too soon, Lechtreck et al. report. The results could lead to new ways of identifying patients prone to fluid buildup in the brain.

Beating cilia circulate cerebrospinal fluid through the brain. If they falter, fluid can accumulate, causing the brain-damaging condition hydrocephalus. Mice carrying mutations in the gene for the protein hydin develop hydrocephalus, but it wasn't known why. Last year, the researchers discovered a clue by finding that the absence of hydin caused flagella in the protist Chlamydomonas to freeze up. Now the team has observed a similar effect in the cilia of mice.

Cilia from mice with Hydin mutations beat abnormally. Instead of showing a smooth back-and-forth movement, the cilia merely vibrated. They also beat more slowly and often stopped. Though the precise function of the hydrin protein is unclear, it is known to be part of the cilia's axoneme. Indeed, the Hydin mice also had a subtle structural defect in their cilia—a knob on one of the central microtubules was absent.

The authors believe that this defect might cause a lack of coordination among the dynein motors that move cilia. Normally, motors on one side of the shaft kick into gear to produce the forward stroke. Then they flip off and motors on the opposite side turn on, producing the return stoke. But the researchers suspect that this switch doesn't occur in hydin-lacking cilia, causing the shaft to relax prematurely. The results predict that humans with defects in hydin will develop hydrocephalus and primary ciliary dyskinesia, an inherited disorder caused by defective airway cilia.

Reference:

Lechtreck, K.-F., et al. 2008. J. Cell Biol. 180:633–643.[Abstract/Free Full Text]

Mitch Leslie

mitchleslie{at}comcast.net

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This Article Full Text (PDF, 1445K) PPT slides of all figures Alert me when this article is cited Services Email this article Similar articles in this journal Alert me to new content in the JCB Download to citation manager Citing Articles Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Leslie, M. Search for Related Content PubMed Articles by Leslie, M. Social Bookmarking                            What's this?