期刊
PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
卷 116, 期 47, 页码 23562-23572出版社
NATL ACAD SCIENCES
DOI: 10.1073/pnas.1902003116
关键词
primary cilia; photoreceptors; ciliopathies; superresolution; cryoelectron tomography
资金
- NIH [R01-EY007981, R01-EY026545, F31-EY028025, F32-EY027171]
- Welch Foundation [Q0035, AU-1714]
- National Center for Macromolecular Imaging [P41-GM103832]
- Baylor College of Medicine [P30-EY002520]
- NATIONAL EYE INSTITUTE [P30EY002520] Funding Source: NIH RePORTER
Primary cilia carry out numerous signaling and sensory functions, and defects in them, ciliopathies, cause a range of symptoms, including blindness. Understanding of their nanometer-scale ciliary substructures and their disruptions in ciliopathies has been hindered by limitations of conventional microscopic techniques. We have combined cryoelectron tomography, enhanced by subtomogram averaging, with superresolution stochastic optical reconstruction microscopy (STORM) to define subdomains within the light-sensing rod sensory cilium of mouse retinas and reveal previously unknown substructures formed by resident proteins. Domains are demarcated by structural features such as the axoneme and its connections to the ciliary membrane, and are correlated with molecular markers of subcompartments, including the lumen and walls of the axoneme, the membrane glycocalyx, and the intervening cytoplasm. Within this framework, we report spatial distributions of key proteins in wild-type (WT) mice and the effects on them of genetic deficiencies in 3 models of Bardet-Biedl syndrome.
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