| SILVA, Ivan Rosa e, Lucia BINÓ, Christopher M. JOHNSON, Trevor J. RUTHERFORD, David NEUHAUS, Antonina ANDREEVA, Lukáš ČAJÁNEK and Mark van BREUGEL. Molecular mechanisms underlying the role of the centriolar CEP164-TTBK2 complex in ciliopathies. Structure. Cambridge: Cell Press, 2022, vol. 30, No 1, p. "114-128"-"e1-e9", 25 pp. ISSN 0969-2126. Available from: https://dx.doi.org/10.1016/j.str.2021.08.007. |
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@article{1818485,
author = {Silva, Ivan Rosa e and Binó, Lucia and Johnson, Christopher M. and Rutherford, Trevor J. and Neuhaus, David and Andreeva, Antonina and Čajánek, Lukáš and Breugel, Mark van},
article_location = {Cambridge},
article_number = {1},
doi = {http://dx.doi.org/10.1016/j.str.2021.08.007},
keywords = {CEP164; TTBK2; ciliaciliogenesis; ciliopathy; nephronophthisis; basal body; centriole; distal appendage; centrosome},
language = {eng},
issn = {0969-2126},
journal = {Structure},
title = {Molecular mechanisms underlying the role of the centriolar CEP164-TTBK2 complex in ciliopathies},
url = {https://www.sciencedirect.com/science/article/pii/S0969212621003026},
volume = {30},
year = {2022}
}
TY - JOUR
ID - 1818485
AU - Silva, Ivan Rosa e - Binó, Lucia - Johnson, Christopher M. - Rutherford, Trevor J. - Neuhaus, David - Andreeva, Antonina - Čajánek, Lukáš - Breugel, Mark van
PY - 2022
TI - Molecular mechanisms underlying the role of the centriolar CEP164-TTBK2 complex in ciliopathies
JF - Structure
VL - 30
IS - 1
SP - "114-128"-"e1-e9"
EP - "114-128"-"e1-e9"
PB - Cell Press
SN - 09692126
KW - CEP164
KW - TTBK2
KW - ciliaciliogenesis
KW - ciliopathy
KW - nephronophthisis
KW - basal body
KW - centriole
KW - distal appendage
KW - centrosome
UR - https://www.sciencedirect.com/science/article/pii/S0969212621003026
N2 - Cilia formation is essential for human life. One of the earliest events in the ciliogenesis program is the recruitment of tau-tubulin kinase 2 (TTBK2) by the centriole distal appendage component CEP164. Due to the lack of high-resolution structural information on this complex, it is unclear how it is affected in human ciliopathies such as nephronophthisis. Furthermore, it is poorly understood if binding to CEP164 influences TTBK2 activities. Here, we present a detailed biochemical, structural, and functional analysis of the CEP164-TTBK2 complex and demonstrate how it is compromised by two ciliopathic mutations in CEP164. Moreover, we also provide insights into how binding to CEP164 is coordinated with TTBK2 activities. Together, our data deepen our understanding of a crucial step in cilia formation and will inform future studies aimed at restoring CEP164 functionality in a debilitating human ciliopathy.
ER -
SILVA, Ivan Rosa e, Lucia BINÓ, Christopher M. JOHNSON, Trevor J. RUTHERFORD, David NEUHAUS, Antonina ANDREEVA, Lukáš ČAJÁNEK and Mark van BREUGEL. Molecular mechanisms underlying the role of the centriolar CEP164-TTBK2 complex in ciliopathies. \textit{Structure}. Cambridge: Cell Press, 2022, vol.~30, No~1, p.~''114-128''-''e1-e9'', 25 pp. ISSN~0969-2126. Available from: https://dx.doi.org/10.1016/j.str.2021.08.007.
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