title: Mammalian axoneme central pair complex proteins: Broader roles revealed by gene knockout phenotypes.
abstract: The axoneme genes, their encoded proteins, their functions and the structures they form are largely conserved across species. Much of our knowledge of the function and structure of axoneme proteins in cilia and flagella is derived from studies on model organisms like the green algae, Chlamydomonas reinhardtii. The core structure of cilia and flagella is the axoneme, which in most motile cilia and flagella contains a 9 + 2 configuration of microtubules. The two central microtubules are the scaffold of the central pair complex (CPC). Mutations that disrupt CPC genes in Chlamydomonas and other model organisms result in defects in assembly, stability and function of the axoneme, leading to flagellar motility defects. However, targeted mutations generated in mice in the orthologous CPC genes have revealed significant differences in phenotypes of mutants compared to Chlamydomonas. Here we review observations that support the concept of cell-type specific roles for the CPC genes in mice, and an expanded repertoire of functions for the products of these genes in cilia, including non-motile cilia, and other microtubule-associated cellular functions.
authors.affiliation: Department of Obstetrics and Gynecology, Virginia Commonwealth University, Richmond, Virginia.
authors.forename: Maria E
authors.initials: ME
authors.lastname: Teves
authors.affiliation: Department of Obstetrics and Gynecology, Virginia Commonwealth University, Richmond, Virginia.|Department of Biochemistry and Molecular Biology, Virginia Commonwealth University, Richmond, Virginia.
authors.forename: David R
authors.initials: DR
authors.lastname: Nagarkatti-Gude
authors.affiliation: Department of Obstetrics and Gynecology, Virginia Commonwealth University, Richmond, Virginia.|Department of Biochemistry and Molecular Biology, Virginia Commonwealth University, Richmond, Virginia.
authors.forename: Zhibing
authors.initials: Z
authors.lastname: Zhang
authors.affiliation: Department of Obstetrics and Gynecology, Virginia Commonwealth University, Richmond, Virginia.|Department of Biochemistry and Molecular Biology, Virginia Commonwealth University, Richmond, Virginia.
authors.forename: Jerome F
authors.initials: JF
authors.lastname: Strauss
mesh_terms: D000818:Animals; D054468:Axoneme / Q000502:physiology*; D003598:Cytoskeletal Proteins / Q000235:genetics*; D055786:Gene Knockout Techniques / Q000379:methods*; D006801:Humans; D051379:Mice; D008868:Microtubule Proteins / Q000235:genetics*; D008869:Microtubule-Associated Proteins / Q000235:genetics*
chemical_list: D003598:Cytoskeletal Proteins; D008868:Microtubule Proteins; D008869:Microtubule-Associated Proteins
keywords: axoneme; central pair complex; cilia; flagella