![Cep55−/− mice exhibit Meckel–Gruber syndrome phenotypes and elongated primary cilia. (A) Coronal and sagittal MRI of Cep55+/+ and Cep55−/− heads at E18.5. Arrowheads indicate hydrocephalus and ventricle dilatations in Cep55−/− embryos compared with the morphology in wild-type embryos. Scale bars, 2 mm. (B and C) E14.5 and E18.5 coronal sections from anterior to posterior through heads and brains, respectively. 3V, third ventricle; CPu, caudate putamen; Cx, cortex; D3V, dorsal third ventricle; Hippo, hippocampus; LV, lateral ventricle; Th, thalamus. Scale bars, 1 mm. (D) P0.5 Cep55+/+ and Cep55−/− CPECs were stained with the ciliary marker (GT335, green) and DNA (blue). Insets show zoomed-in views of the boxed regions. Scale bars, 5 µm (main image) and 1 µm (magnified region). (E) Quantitative analysis of the cilium length in D (four mice per group). (F) Representative images of mid-sagittal view of the brain from Cep55+/+ and Cep55−/− littermates at P0.5. The dashed boxes indicate cerebellum. Scale bar, 1 mm. (G) The ratio of midline cerebellum area (null/wild type [wt]) quantified in ImageJ software from Cep55+/+ and Cep55−/− littermates (four mice per group). (H) Scanning electron micrographs show that primary cilia in E10.5 Cep55−/− neural tube are bulged at the bases and shafts of cilia (arrowheads). Scale bar, 2 µm. (I) E18.5 transverse sections of the kidneys from Cep55+/+ and Cep55−/− littermates were stained with hematoxylin and eosin. gl, glomerulus; rt, renal tubule. Insets show zoomed-in views of the boxed regions. Scale bars, 100 µm (main image) and 50 µm (magnified region). (J) E18.5 Cep55+/+ and Cep55−/− kidney sections were stained with the ciliary marker (Ac-tubulin, green) and DNA (blue). The dashed circles indicate renal tubules. Scale bar, 10 µm. (K) Quantitative analysis of the cilium length in H (four mice per group). (L) Summarized and quantified results of four main MKS phenotypes in Cep55−/− mice at P2 or E18.5. Data are means ± SD of three independent experiments in E, G, and K. An unpaired two-tailed t test was performed. ***, P < 0.001.](https://cdn.rupress.org/rup/content_public/journal/jcb/220/2/10.1083_jcb.202003149/4/jcb_202003149_fig1.png?Expires=1767804679&Signature=Wy2UydAzHlxrLNjo0XoJ~ZIn7T0VPO6cy2ikA-5Cd0sazY0fUIOd9RvEC4ANMo53eu8aTO9b4Q1YVwSsRkx1S0-qbwb6XpDZSqJQTDluCrhom9kSrlLNLZmSNXpegAfc4tU9UGJ5NlJe6E43sb84EhoFOPqHzwzAsRoswvnMVNydrkEMdUsK4FOd~Rr0bI18uiHDefwDFANhMABRUgHXIJ4FkF7x9QKq9d3qDXUkDKgC6vTcg232Bnkh4cM0jcpUZi~TYvTvmUuzAZfzIMF5~Batv6vlaAhRpfXFV2BrzjuUi7D867OClcR0UcDVemTsQ~OJQfGDrFZ-bWBkUPxIdw__&Key-Pair-Id=APKAIE5G5CRDK6RD3PGA)
Cep55−/− mice exhibit Meckel–Gruber syndrome phenotypes and elongated primary cilia. (A) Coronal and sagittal MRI of Cep55+/+ and Cep55−/− heads at E18.5. Arrowheads indicate hydrocephalus and ventricle dilatations in Cep55−/− embryos compared with the morphology in wild-type embryos. Scale bars, 2 mm. (B and C) E14.5 and E18.5 coronal sections from anterior to posterior through heads and brains, respectively. 3V, third ventricle; CPu, caudate putamen; Cx, cortex; D3V, dorsal third ventricle; Hippo, hippocampus; LV, lateral ventricle; Th, thalamus. Scale bars, 1 mm. (D) P0.5 Cep55+/+ and Cep55−/− CPECs were stained with the ciliary marker (GT335, green) and DNA (blue). Insets show zoomed-in views of the boxed regions. Scale bars, 5 µm (main image) and 1 µm (magnified region). (E) Quantitative analysis of the cilium length in D (four mice per group). (F) Representative images of mid-sagittal view of the brain from Cep55+/+ and Cep55−/− littermates at P0.5. The dashed boxes indicate cerebellum. Scale bar, 1 mm. (G) The ratio of midline cerebellum area (null/wild type [wt]) quantified in ImageJ software from Cep55+/+ and Cep55−/− littermates (four mice per group). (H) Scanning electron micrographs show that primary cilia in E10.5 Cep55−/− neural tube are bulged at the bases and shafts of cilia (arrowheads). Scale bar, 2 µm. (I) E18.5 transverse sections of the kidneys from Cep55+/+ and Cep55−/− littermates were stained with hematoxylin and eosin. gl, glomerulus; rt, renal tubule. Insets show zoomed-in views of the boxed regions. Scale bars, 100 µm (main image) and 50 µm (magnified region). (J) E18.5 Cep55+/+ and Cep55−/− kidney sections were stained with the ciliary marker (Ac-tubulin, green) and DNA (blue). The dashed circles indicate renal tubules. Scale bar, 10 µm. (K) Quantitative analysis of the cilium length in H (four mice per group). (L) Summarized and quantified results of four main MKS phenotypes in Cep55−/− mice at P2 or E18.5. Data are means ± SD of three independent experiments in E, G, and K. An unpaired two-tailed t test was performed. ***, P < 0.001.