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The Ciliary Cytoskeleton

Lotte B. Pedersen, Jacob M. Schrøder, Peter Satir, Søren T. Christensen

10.1002/cphy.c110043

Source: Volume 2, Issue 1, January 2012

Published online: January 2012

Full Article on Wiley Online Library



Abstract

Cilia and flagella are surface‐exposed, finger‐like organelles whose core consists of a microtubule (MT)‐based axoneme that grows from a modified centriole, the basal body. Cilia are found on the surface of many eukaryotic cells and play important roles in cell motility and in coordinating a variety of signaling pathways during growth, development, and tissue homeostasis. Defective cilia have been linked to a number of developmental disorders and diseases, collectively called ciliopathies. Cilia are dynamic organelles that assemble and disassemble in tight coordination with the cell cycle. In most cells, cilia are assembled during growth arrest in a multistep process involving interaction of vesicles with appendages present on the distal end of mature centrioles, and addition of tubulin and other building blocks to the distal tip of the basal body and growing axoneme; these building blocks are sorted through a region at the cilium base known as the ciliary necklace, and then transported via intraflagellar transport (IFT) along the axoneme toward the tip for assembly. After assembly, the cilium frequently continues to turn over and incorporate tubulin at its distal end in an IFT‐dependent manner. Prior to cell division, the cilia are usually resorbed to liberate centrosomes for mitotic spindle pole formation. Here, we present an overview of the main cytoskeletal structures associated with cilia and centrioles with emphasis on the MT‐associated appendages, fibers, and filaments at the cilium base and tip. The composition and possible functions of these structures are discussed in relation to cilia assembly, disassembly, and length regulation. © 2012 American Physiological Society. Compr Physiol 2:779‐803, 2012.

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Figure 1. Figure 1.

Examples of different types of cilia. (A‐D) Examples of motile cilia. (A) Digital interference contrast (DIC) image of the green alga Chlamydomonas reinhardtii with two motile cilia/flagella. Adapted from 233, with permission, from Elsevier. (B) Schematic cross section of a 9+2 motile cilium with inner (green) and outer dynein arms (red), radial spokes (light blue), nexin links (dark gray), and a central MT pair surrounded by an inner sheath (light gray). (C) Scanning electron micrograph (SEM) of mouse tracheal cilia [courtesy of Karl F. Lechtreck and George B. Witman, and reproduced from 233, with permission from Elsevier]. (D) DIC image of a human sperm cell with one motile flagellum. (E‐H) Examples of immotile primary cilia. (E) Immunofluorescence micrograph of a human foreskin fibroblast (hFF) stained with antibodies against detyrosinated tubulin (green) marking the axoneme, against dynactin subunit p150Glued (red) that label the centrosome, and 4′,6‐diamidino‐2‐phenylindole (DAPI), which labels the DNA (blue). (F) Schematic cross section of a 9+0 cilium with the nine outer doublets and nexin links (dark gray). (G) SEM of a cultured IMCD cell with a primary cilium. Note the bulged appearance of the distal cilium tip (courtesy of Alexandre Benmerah, Phillipe Bastin, and Thierry Blisnick). (H) Transmission electron micrograph of a longitudinal section of a primary cilium of an hFF cell [adapted from 275, with permission from Journal of Cell Science].
Figure 2. Figure 2.

Structure of the basal body, transition zone, and ciliary axoneme. (A) Schematic longitudinal view of a motile cilium with the 9+2 axoneme extending from the nine triplet structure of the basal body, which is linked to the daughter centriole by the rootlet filaments. The ciliary axoneme is surrounded by the ciliary membrane. (B, F) Cross section of a 9+2 cilium with outer (ODA) and inner dynein arms (IDA), radial spokes (RS) and a central MT pair surrounded by an inner sheath. The outer doublets are connected by nexin links. TEM of a cross‐sectioned Chlamydomonas flagellum (F) is courtesy of Stefan Geimer, University of Bayreuth. (C, G) Cross section of the ciliary necklace region, with Y‐links connecting the A subfiber of the outer doublet MTs to the ciliary membrane [TEM is from 109, with permission from Journal of Cell Science]. (D, H) Cross section of the distal part of the basal body. The transitional fibers are attached in a rotational asymmetric pattern to all three MT subfibers of the basal body. (E, I) Cross section of the nine triplet structures of the basal body [TEM image is from 102, with permission from Journal of Cell Science].
Figure 3. Figure 3.

Appendages of centrioles and the basal body. (A) Schematic model of an early G1 centriole pair with their proximal regions connected by a linker structure of rootlet filaments. The older (mother) centriole is the uppermost and can be distinguished from the daughter by the presence of distal‐ and subdistal appendages. The centriole pair is embedded in a matrix of pericentriolar material (PCM). (B) The cilium is nucleated from the distal region of the basal body, and the transition fibers, corresponding to the distal appendages of the mother centriole, extend from the basal body and connect to the plasma membrane at the cilium base (see Fig. 2A). Above the transition fibers, the Y‐links of the necklace are present.
Figure 4. Figure 4.

Cilia and the cell cycle. Assembly and disassembly of primary cilia are tightly coordinated with the cell cycle. In G1/G0 the mother centriole docks at the apical membrane at the site of ciliary assembly and the primary cilium is nucleated. The elongation of the distal end of the mother centriole is mediated by IFT‐dependent addition of ciliary precursors, and the mother centriole becomes the basal body. The disassembly of the primary cilium prior to mitosis liberates both centriole pairs to function in mitotic spindle formation. See text for details.
Figure 5. Figure 5.

Early stages of primary cilia assembly. (A) In the intracellular pathway, a centriolar vesicle localizes to the distal end of the mother centriole and the axoneme then elongates within this vesicle while nearby vesicles fuse to form a sheath surrounding the axonemal shaft. The sheath eventually reaches and fuses with the plasma membrane, and the distal part of the cilium is in contact with the extracellular milieu while the proximal part is surrounded by a ciliary pocket in the cytoplasm. (B) In the extracellular pathway, the mother centriole docks directly to the plasma membrane, and most of the cilium protrude out into the extracellular milieu. The figure is based on: 202,293,294,321.
Figure 6. Figure 6.

IFT and targeting of proteins to the cilium. Axonemal precursors, as well as membrane proteins, are transported along MTs to the primary cilium via Golgi‐derived vesicles. At the ciliary base the vesicles are exocytosed and the ciliary proteins associate with IFT particles, and enter the ciliary compartment through the transition zone. After entry, kinesin‐2 transports these proteins, as well as cytoplasmic dynein 2, along the axoneme to the ciliary tip (anterograde transport). At the ciliary tip, kinesin‐2 is inactivated and cytoplasmic dynein 2 is activated and brings IFT particles and ciliary turnover products (e.g., inactive receptors) along the axoneme back to the cell body (retrograde transport) for recycling or degradation. Modified, with permission, from 233. See text for further details.
Figure 7. Figure 7.

The ciliary tip. (A, B) Structures associated with the ciliary tip. (A) Whole mount electron micrograph of the distal tips of Chlamydomonas flagella, treated with detergent. Arrows indicate the distal filaments attached to the end of A subfibers [reproduced from 77, with permission from Journal of Cell Biology]. (B) Schematic model of the flagellar tip structures showing filaments extending from the tip of A tubule of the MT doublets to the membrane, as well as the cap structure on the distal end of the central pair MTs. (C) Immunofluorescence micrograph of a human bronchial epithelial cell stained with antibodies against EB3 (green) and acetylated alpha tubulin (red) to label cilia, and DAPI (blue) to visualize DNA. EB3 localizes to the tip as well as the base of the motile cilia [adapted from 275, with permission from Journal of Cell Science]. Scalebar, 5 μm. (D) Immunofluorescence micrograph of a Chlamydomonas cell labeled with antibodies specific for CrEB1 (red) 231 and acetylated alpha tubulin (green). The upper left insert is an enlarged shifted overlay showing the flagellar tip localization of CrEB1. Scalebar, 5 μm.


Figure 1.

Examples of different types of cilia. (A‐D) Examples of motile cilia. (A) Digital interference contrast (DIC) image of the green alga Chlamydomonas reinhardtii with two motile cilia/flagella. Adapted from 233, with permission, from Elsevier. (B) Schematic cross section of a 9+2 motile cilium with inner (green) and outer dynein arms (red), radial spokes (light blue), nexin links (dark gray), and a central MT pair surrounded by an inner sheath (light gray). (C) Scanning electron micrograph (SEM) of mouse tracheal cilia [courtesy of Karl F. Lechtreck and George B. Witman, and reproduced from 233, with permission from Elsevier]. (D) DIC image of a human sperm cell with one motile flagellum. (E‐H) Examples of immotile primary cilia. (E) Immunofluorescence micrograph of a human foreskin fibroblast (hFF) stained with antibodies against detyrosinated tubulin (green) marking the axoneme, against dynactin subunit p150Glued (red) that label the centrosome, and 4′,6‐diamidino‐2‐phenylindole (DAPI), which labels the DNA (blue). (F) Schematic cross section of a 9+0 cilium with the nine outer doublets and nexin links (dark gray). (G) SEM of a cultured IMCD cell with a primary cilium. Note the bulged appearance of the distal cilium tip (courtesy of Alexandre Benmerah, Phillipe Bastin, and Thierry Blisnick). (H) Transmission electron micrograph of a longitudinal section of a primary cilium of an hFF cell [adapted from 275, with permission from Journal of Cell Science].



Figure 2.

Structure of the basal body, transition zone, and ciliary axoneme. (A) Schematic longitudinal view of a motile cilium with the 9+2 axoneme extending from the nine triplet structure of the basal body, which is linked to the daughter centriole by the rootlet filaments. The ciliary axoneme is surrounded by the ciliary membrane. (B, F) Cross section of a 9+2 cilium with outer (ODA) and inner dynein arms (IDA), radial spokes (RS) and a central MT pair surrounded by an inner sheath. The outer doublets are connected by nexin links. TEM of a cross‐sectioned Chlamydomonas flagellum (F) is courtesy of Stefan Geimer, University of Bayreuth. (C, G) Cross section of the ciliary necklace region, with Y‐links connecting the A subfiber of the outer doublet MTs to the ciliary membrane [TEM is from 109, with permission from Journal of Cell Science]. (D, H) Cross section of the distal part of the basal body. The transitional fibers are attached in a rotational asymmetric pattern to all three MT subfibers of the basal body. (E, I) Cross section of the nine triplet structures of the basal body [TEM image is from 102, with permission from Journal of Cell Science].



Figure 3.

Appendages of centrioles and the basal body. (A) Schematic model of an early G1 centriole pair with their proximal regions connected by a linker structure of rootlet filaments. The older (mother) centriole is the uppermost and can be distinguished from the daughter by the presence of distal‐ and subdistal appendages. The centriole pair is embedded in a matrix of pericentriolar material (PCM). (B) The cilium is nucleated from the distal region of the basal body, and the transition fibers, corresponding to the distal appendages of the mother centriole, extend from the basal body and connect to the plasma membrane at the cilium base (see Fig. 2A). Above the transition fibers, the Y‐links of the necklace are present.



Figure 4.

Cilia and the cell cycle. Assembly and disassembly of primary cilia are tightly coordinated with the cell cycle. In G1/G0 the mother centriole docks at the apical membrane at the site of ciliary assembly and the primary cilium is nucleated. The elongation of the distal end of the mother centriole is mediated by IFT‐dependent addition of ciliary precursors, and the mother centriole becomes the basal body. The disassembly of the primary cilium prior to mitosis liberates both centriole pairs to function in mitotic spindle formation. See text for details.



Figure 5.

Early stages of primary cilia assembly. (A) In the intracellular pathway, a centriolar vesicle localizes to the distal end of the mother centriole and the axoneme then elongates within this vesicle while nearby vesicles fuse to form a sheath surrounding the axonemal shaft. The sheath eventually reaches and fuses with the plasma membrane, and the distal part of the cilium is in contact with the extracellular milieu while the proximal part is surrounded by a ciliary pocket in the cytoplasm. (B) In the extracellular pathway, the mother centriole docks directly to the plasma membrane, and most of the cilium protrude out into the extracellular milieu. The figure is based on: 202,293,294,321.



Figure 6.

IFT and targeting of proteins to the cilium. Axonemal precursors, as well as membrane proteins, are transported along MTs to the primary cilium via Golgi‐derived vesicles. At the ciliary base the vesicles are exocytosed and the ciliary proteins associate with IFT particles, and enter the ciliary compartment through the transition zone. After entry, kinesin‐2 transports these proteins, as well as cytoplasmic dynein 2, along the axoneme to the ciliary tip (anterograde transport). At the ciliary tip, kinesin‐2 is inactivated and cytoplasmic dynein 2 is activated and brings IFT particles and ciliary turnover products (e.g., inactive receptors) along the axoneme back to the cell body (retrograde transport) for recycling or degradation. Modified, with permission, from 233. See text for further details.



Figure 7.

The ciliary tip. (A, B) Structures associated with the ciliary tip. (A) Whole mount electron micrograph of the distal tips of Chlamydomonas flagella, treated with detergent. Arrows indicate the distal filaments attached to the end of A subfibers [reproduced from 77, with permission from Journal of Cell Biology]. (B) Schematic model of the flagellar tip structures showing filaments extending from the tip of A tubule of the MT doublets to the membrane, as well as the cap structure on the distal end of the central pair MTs. (C) Immunofluorescence micrograph of a human bronchial epithelial cell stained with antibodies against EB3 (green) and acetylated alpha tubulin (red) to label cilia, and DAPI (blue) to visualize DNA. EB3 localizes to the tip as well as the base of the motile cilia [adapted from 275, with permission from Journal of Cell Science]. Scalebar, 5 μm. (D) Immunofluorescence micrograph of a Chlamydomonas cell labeled with antibodies specific for CrEB1 (red) 231 and acetylated alpha tubulin (green). The upper left insert is an enlarged shifted overlay showing the flagellar tip localization of CrEB1. Scalebar, 5 μm.

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Article Title: The Ciliary Cytoskeleton
 

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Lotte B. Pedersen, Jacob M. Schrøder, Peter Satir, Søren T. Christensen. The Ciliary Cytoskeleton. Compr Physiol 2012, 2: 779-803. doi: 10.1002/cphy.c110043