Mechanisms of mammalian ciliary motility: Insights from primary ciliary dyskinesia genetics

Gene. 2011 Mar 1;473(2):57-66. doi: 10.1016/j.gene.2010.11.006. Epub 2010 Nov 25.

Abstract

Motile cilia and flagella are organelles that, historically, have been poorly understood and inadequately investigated. However, cilia play critical roles in fluid clearance in the respiratory system and the brain, and flagella are required for sperm motility. Genetic studies involving human patients and mouse models of primary ciliary dyskinesia over the last decade have uncovered a number of important ciliary proteins and have begun to elucidate the mechanisms underlying ciliary motility. When combined with genetic, biochemical, and cell biological studies in Chlamydomonas reinhardtii, these mammalian genetic analyses begin to reveal the mechanisms by which ciliary motility is regulated.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Chlamydomonas reinhardtii / genetics
  • Cilia / genetics*
  • Cilia / ultrastructure
  • Ciliary Motility Disorders / genetics*
  • Disease Models, Animal
  • Dyneins / genetics
  • Flagella / genetics
  • Flagella / ultrastructure
  • Humans
  • Kartagener Syndrome / genetics
  • Mice
  • Transcription Factors / physiology

Substances

  • Transcription Factors
  • Dyneins