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Mutations in Hydin impair ciliary motility in mice

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 c...

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Autores principales: Lechtreck, Karl-Ferdinand, Delmotte, Philippe, Robinson, Michael L., Sanderson, Michael J., Witman, George B.
Formato: Texto
Lenguaje:English
Publicado: The Rockefeller University Press 2008
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2234243/
https://www.ncbi.nlm.nih.gov/pubmed/18250199
http://dx.doi.org/10.1083/jcb.200710162
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author Lechtreck, Karl-Ferdinand
Delmotte, Philippe
Robinson, Michael L.
Sanderson, Michael J.
Witman, George B.
author_facet Lechtreck, Karl-Ferdinand
Delmotte, Philippe
Robinson, Michael L.
Sanderson, Michael J.
Witman, George B.
author_sort Lechtreck, Karl-Ferdinand
collection PubMed
description 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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spelling pubmed-22342432008-08-11 Mutations in Hydin impair ciliary motility in mice Lechtreck, Karl-Ferdinand Delmotte, Philippe Robinson, Michael L. Sanderson, Michael J. Witman, George B. J Cell Biol Research Articles 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. The Rockefeller University Press 2008-02-11 /pmc/articles/PMC2234243/ /pubmed/18250199 http://dx.doi.org/10.1083/jcb.200710162 Text en Copyright © 2008, The Rockefeller University Press This article is distributed under the terms of an Attribution–Noncommercial–Share Alike–No Mirror Sites license for the first six months after the publication date (see http://www.rupress.org/terms). After six months it is available under a Creative Commons License (Attribution–Noncommercial–Share Alike 4.0 Unported license, as described at http://creativecommons.org/licenses/by-nc-sa/4.0/).
spellingShingle Research Articles
Lechtreck, Karl-Ferdinand
Delmotte, Philippe
Robinson, Michael L.
Sanderson, Michael J.
Witman, George B.
Mutations in Hydin impair ciliary motility in mice
title Mutations in Hydin impair ciliary motility in mice
title_full Mutations in Hydin impair ciliary motility in mice
title_fullStr Mutations in Hydin impair ciliary motility in mice
title_full_unstemmed Mutations in Hydin impair ciliary motility in mice
title_short Mutations in Hydin impair ciliary motility in mice
title_sort mutations in hydin impair ciliary motility in mice
topic Research Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2234243/
https://www.ncbi.nlm.nih.gov/pubmed/18250199
http://dx.doi.org/10.1083/jcb.200710162
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