Cilium transition zone proteome reveals compartmentalization and differential dynamics of ciliopathy complexes
The result's identifiers
Result code in IS VaVaI
<a href="https://www.isvavai.cz/riv?ss=detail&h=RIV%2F68378050%3A_____%2F16%3A00473106" target="_blank" >RIV/68378050:_____/16:00473106 - isvavai.cz</a>
Result on the web
<a href="http://dx.doi.org/10.1073/pnas.1604258113" target="_blank" >http://dx.doi.org/10.1073/pnas.1604258113</a>
DOI - Digital Object Identifier
<a href="http://dx.doi.org/10.1073/pnas.1604258113" target="_blank" >10.1073/pnas.1604258113</a>
Alternative languages
Result language
angličtina
Original language name
Cilium transition zone proteome reveals compartmentalization and differential dynamics of ciliopathy complexes
Original language description
The transition zone (TZ) of eukaryotic cilia and flagella is a structural intermediate between the basal body and the axoneme that regulates ciliary traffic. Mutations in genes encoding TZ proteins (TZPs) cause human inherited diseases (ciliopathies). Here, we use the trypanosome to identify TZ components and localize them to TZ subdomains, showing that the Bardet-Biedl syndrome complex (BBSome) is more distal in the TZ than the Meckel syndrome (MKS) complex. Several of the TZPs identified here have human orthologs. Functional analysis shows essential roles for TZPs in motility, in building the axoneme central pair apparatus and in flagellum biogenesis. Analysis using RNAi and HaloTag fusion protein approaches reveals that most TZPs (including the MKS ciliopathy complex) show long-term stable association with the TZ, whereas the BBSome is dynamic. We propose that some Bardet-Biedl syndrome and MKS pleiotropy may be caused by mutations that impact TZP complex dynamics.
Czech name
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Czech description
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Classification
Type
J<sub>x</sub> - Unclassified - Peer-reviewed scientific article (Jimp, Jsc and Jost)
CEP classification
EB - Genetics and molecular biology
OECD FORD branch
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Result continuities
Project
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Continuities
I - Institucionalni podpora na dlouhodoby koncepcni rozvoj vyzkumne organizace
Others
Publication year
2016
Confidentiality
S - Úplné a pravdivé údaje o projektu nepodléhají ochraně podle zvláštních právních předpisů
Data specific for result type
Name of the periodical
Proceedings of the National Academy of Sciences of the United States of America
ISSN
0027-8424
e-ISSN
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Volume of the periodical
113
Issue of the periodical within the volume
35
Country of publishing house
US - UNITED STATES
Number of pages
9
Pages from-to
"E5135"-"E5143"
UT code for WoS article
000383090700009
EID of the result in the Scopus database
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