Summary of 206-Miyamoto-CiliaryMorphology

SSBD:database URL
3D FIB-SEM images of a cross section of the ciliary pocket membrane in hTERT-RPE1 cells.
Relase date
Updated date
Image data based on Experiment
Number of Datasets
1 ( Image datasets: 1, Quantitative data datasets: 0 )
Size of Datasets
173.4 MB ( Image datasets: 173.4 MB, Quantitative data datasets: 0 bytes )

Homo sapiens
Cell lines(s)
hTERT RPE-1 cell

Molecular Function (MF)
Biological Process (BP)
Cellular Component (CC)
ciliary pocket membrane, peroxisome
Biological Imaging Method
scanning electron microscopy
X scale
4.13 nanometer/pixel
Y scale
5.25 nanometer/pixel
Z scale
10 nanometer/slice
T scale

Image Acquisition
Experiment type
Microscope type
Acquisition mode
Contrast method
Microscope model
Detector model
Objective model
Filter set

Related paper(s)

Tatsuo Miyamoto, Kosuke Hosoba, Takeshi Itabashi, Atsuko H Iwane, Silvia Natsuko Akutsu, Hiroshi Ochiai, Yumiko Saito, Takashi Yamamoto, Shinya Matsuura (2020) Insufficiency of ciliary cholesterol in hereditary Zellweger syndrome., The EMBO journal, Volume 39, Number 12, pp. e103499

Published in 2020 Jun 17 (Electronic publication in May 5, 2020, midnight )

(Abstract) Primary cilia are antenna-like organelles on the surface of most mammalian cells that receive sonic hedgehog (Shh) signaling in embryogenesis and carcinogenesis. Cellular cholesterol functions as a direct activator of a seven-transmembrane oncoprotein called Smoothened (Smo) and thereby induces Smo accumulation on the ciliary membrane where it transduces the Shh signal. However, how cholesterol is supplied to the ciliary membrane remains unclear. Here, we report that peroxisomes are essential for the transport of cholesterol into the ciliary membrane. Zellweger syndrome (ZS) is a peroxisome-deficient hereditary disorder with several ciliopathy-related features and cells from these patients showed a reduced cholesterol level in the ciliary membrane. Reverse genetics approaches revealed that the GTP exchange factor Rabin8, the Rab GTPase Rab10, and the microtubule minus-end-directed kinesin KIFC3 form a peroxisome-associated complex to control the movement of peroxisomes along microtubules, enabling communication between peroxisomes and ciliary pocket membranes. Our findings suggest that insufficient ciliary cholesterol levels may underlie ciliopathies.
(MeSH Terms)

Tatsuo Miyamoto, Shinya Matsuura , Hiroshima University, Hiroshima University , Department of Genetics and Cell Biology, Research Institute for Radiation Biology and Medicine, Department of Genetics and Cell Biology, Research Institute for Radiation Biology and Medicine

Dataset List of 206-Miyamoto-CiliaryMorphology

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# 7533
Dataset Kind Image data
Dataset Size 173.4 MB
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