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Review
. 2015 Jul;48(7):380-7.
doi: 10.5483/bmbrep.2015.48.7.084.

Ciliary subcompartments: how are they established and what are their functions?

Affiliations
Review

Ciliary subcompartments: how are they established and what are their functions?

Jeongmi Lee et al. BMB Rep. 2015 Jul.

Abstract

Cilia are conserved subcellular organelles with diverse sensory and developmental roles. Recently, they have emerged as crucial organelles whose dysfunction causes a wide spectrum of disorders called ciliopathies. Recent studies on the pathological mechanisms underlying ciliopathies showed that the ciliary compartment is further divided into subdomains with specific roles in the biogenesis, maintenance and function of cilia. Several conserved sets of molecules that play specific roles in each subcompartment have been discovered. Here we review recent progress on our understanding of ciliary subcompartments, especially focusing on the molecules required for their structure and/or function.

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Figures

Fig. 1
Fig. 1. Model of early ciliogenesis. Rab11-positive recycling endosomes trafficked toward the mother centriole-associated distal appendages (DA). When the vesicles dock into the DA, they become distal appendage vesicles (DAVs). EHD1 included in the DAVs triggers SNARE-dependent membrane fusion to form the ciliary vesicle (CV). EHD1 also triggers transition of the mother centriole to the basal body (BB) by removing CP110 from the mother centriole. After the formation of CV and BB, small GTPase Rab8 is accumulated in the CV and is activated by Rabin8 from Rab11, facilitating elongation of the CV and axoneme. In addition, IFT20 and TZ proteins are delivered to the CV by Golgi-derived vesicles. During elongation of the CV and axoneme, the TZ is formed and the CV membranes are differentiated into the ciliary membrane (orange) and the ciliary sheath (green). Finally, the cilium is formed by the fusion of the ciliary sheath with the apical plasma membrane. The ciliary sheath becomes the periciliary membrane (green).
Fig. 2.
Fig. 2.. Schematic drawing of cilia from different organisms. (A) A typical mammalian primary cilium. The basal body (BB) and the associated distal appendage (DA), the transition zone (TZ), the INV compartment, and the distal tip are shown. The BB microtubules show a typical triplet structure with attached transition fibers (= distal appendage (DA)). In the TZ, the characteristic Y linkers are shown. Some other structures, including the ciliary pocket and the ciliary necklace are also shown. (B) A canonical C. elegans amphid sensory cilium. Ciliary microtubules extend from a fully degenerated basal body at the ciliary base region called the periciliary membrane compartment (PCMC). In the transition zone (TZ), each microtubule doublet is connected to the ciliary membrane via Y-links. Some inner singlet microtubules are also seen. The TZ is followed by the middle segment consisting of 9 doublet microtubules. In the distal segment, the B-tubule of each doublet is missing. (C) A Drosophila chordotonal sensory cilium. The gross structure is similar to that of mammalian cilium. Three distinct features are shown. First, a prominent ciliary dilation (CD) is seen in the middle of the cilium. Second, the axoneme of the proximal segment located between the TZ and the CD contains outer dynein arms. Third, the distal segment contains doublet microtubules without attached outer dynein arms. Color coding: periciliary membrane in green, ciliary membrane in orange.

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