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Figure 4 | Fluids and Barriers of the CNS

Figure 4

From: Structural defects in cilia of the choroid plexus, subfornical organ and ventricular ependyma are associated with ventriculomegaly

Figure 4

BBS mutant mice exhibit structural defects in choroid plexus epithelial cilia. TEM micrographs show normal multiple basal bodies and basal feet in P9 Bbs1M390R/M390R mouse choroid plexus cilia clusters (A). P0 Bbs1M390R/M390R cilia have intact Y-links of the ciliary necklace that link the microtubule doublets and the ciliary membrane in the region of the transition zone (TZ) of the ciliary necklace between the basal body and axoneme (B). Transition fibers (TF) are also seen connecting the basal body to the epithelial plasma membrane. Fifteen month-old Bbs6−/− primary cilia clusters show typical variations of the 9 + 0 mictrotubule arrangement of the axoneme distal to the basal body (arrows; C, D). Longitudinal TEM micrographs of choroid plexus cilia clusters from P0 wild- type (E) and Bbs1M390R/M390R mice (F), P2 Bbs1 mutant mice (G), and P10 Bbs1 mutant mice (H) show disruption along the elongating ciliary axoneme as well as vesicle-like inclusions and electron-dense material that are larger than IFT particles along the ciliary shaft in a sub-population of cilia. In some cilia, the electron-dense material appears between the ciliary plasma membrane and the axoneme (F, G) while in others it is found in the lumen of the cilium, surrounded by the peripheral microtubule doublets. Arrows point to IFT-like particles (F-H). Arrowheads point to large electron-dense, vesicle-like material (F-H). (Bars, 0.5 μm (A), 0.2 μm (B), 0.5 μm (C), 0.2 μm (D), 0.5 μm (E-H).

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