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Three-dimensional architecture of the rod sensory cilium and its disruption in retinal neurodegeneration.
- Source :
-
Cell [Cell] 2012 Nov 21; Vol. 151 (5), pp. 1029-41. - Publication Year :
- 2012
-
Abstract
- Defects in primary cilia lead to devastating disease because of their roles in sensation and developmental signaling but much is unknown about ciliary structure and mechanisms of their formation and maintenance. We used cryo-electron tomography to obtain 3D maps of the connecting cilium and adjacent cellular structures of a modified primary cilium, the rod outer segment, from wild-type and genetically defective mice. The results reveal the molecular architecture of the cilium and provide insights into protein functions. They suggest that the ciliary rootlet is involved in cellular transport and stabilizes the axoneme. A defect in the BBSome membrane coat caused defects in vesicle targeting near the base of the cilium. Loss of the proteins encoded by the Cngb1 gene disrupted links between the disk and plasma membranes. The structures of the outer segment membranes support a model for disk morphogenesis in which basal disks are enveloped by the plasma membrane.<br /> (Copyright © 2012 Elsevier Inc. All rights reserved.)
- Subjects :
- Animals
Cell Membrane metabolism
Cilia chemistry
Cyclic Nucleotide-Gated Cation Channels metabolism
Cytoskeletal Proteins metabolism
Disease Models, Animal
Eye Proteins metabolism
Mice
Mice, Inbred C57BL
Mice, Knockout
Microtubule-Associated Proteins metabolism
Nerve Tissue Proteins metabolism
Retina chemistry
Retina metabolism
Rod Cell Outer Segment chemistry
Rod Cell Outer Segment metabolism
Transport Vesicles metabolism
Cilia ultrastructure
Retinal Diseases pathology
Rod Cell Outer Segment ultrastructure
Subjects
Details
- Language :
- English
- ISSN :
- 1097-4172
- Volume :
- 151
- Issue :
- 5
- Database :
- MEDLINE
- Journal :
- Cell
- Publication Type :
- Academic Journal
- Accession number :
- 23178122
- Full Text :
- https://doi.org/10.1016/j.cell.2012.10.038