The BBSome assembly is spatially controlled by BBS1 and BBS4 in human cells
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F68378050%3A_____%2F20%3A00536141" target="_blank" >RIV/68378050:_____/20:00536141 - isvavai.cz</a>
Result on the web
<a href="https://www.jbc.org/content/295/42/14279" target="_blank" >https://www.jbc.org/content/295/42/14279</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1074/jbc.RA120.013905" target="_blank" >10.1074/jbc.RA120.013905</a>
Alternative languages
Result language
angličtina
Original language name
The BBSome assembly is spatially controlled by BBS1 and BBS4 in human cells
Original language description
Bardet-Biedl syndrome (BBS) is a pleiotropic ciliopathy caused by dysfunction of primary cilia. More than half of BBS patients carry mutations in one of eight genes encoding for subunits of a protein complex, the BBSome, which mediates trafficking of ciliary cargoes. In this study, we elucidated the mechanisms of the BBSome assembly in living cells and how this process is spatially regulated. We generated a large library of human cell lines deficient in a particular BBSome subunit and expressing another subunit tagged with a fluorescent protein. We analyzed these cell lines utilizing biochemical assays, conventional and expansion microscopy, and quantitative fluorescence microscopy techniques: fluorescence recovery after photobleaching and fluorescence correlation spectroscopy. Our data revealed that the BBSome formation is a sequential process. We show that the pre-BBSome is nucleated by BBS4 and assembled at pericentriolar satellites, followed by the translocation of the BBSome into the ciliary base mediated by BBS1. Our results provide a framework for elucidating how BBS-causative mutations interfere with the biogenesis of the BBSome.
Czech name
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Czech description
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Classification
Type
J<sub>imp</sub> - Article in a specialist periodical, which is included in the Web of Science database
CEP classification
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OECD FORD branch
10601 - Cell biology
Result continuities
Project
<a href="/en/project/GJ17-20613Y" target="_blank" >GJ17-20613Y: BBSome and actin interplay in ciliogenesis and formation of the immunological synapse</a><br>
Continuities
I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace
Others
Publication year
2020
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
Journal of Biological Chemistry
ISSN
0021-9258
e-ISSN
1083-351X
Volume of the periodical
295
Issue of the periodical within the volume
42
Country of publishing house
US - UNITED STATES
Number of pages
12
Pages from-to
14279-14290
UT code for WoS article
000586428700004
EID of the result in the Scopus database
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