View source for 7o06


==Crystal structure of the N-terminal domain of CEP164(1-109) bound to camelid nanobody 10Z==
<StructureSection load='7o06' size='340' side='right'caption='[[7o06]], [[Resolution|resolution]] 1.60&Aring;' scene=''>
== Structural highlights ==
<table><tr><td colspan='2'>[[7o06]] is a 4 chain structure with sequence from [https://en.wikipedia.org/wiki/Camelidae_mixed_library Camelidae mixed library] and [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=7O06 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=7O06 FirstGlance]. <br>
</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 1.6&#8491;</td></tr>
<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=7o06 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=7o06 OCA], [https://pdbe.org/7o06 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=7o06 RCSB], [https://www.ebi.ac.uk/pdbsum/7o06 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=7o06 ProSAT]</span></td></tr>
</table>
== Disease ==
[https://www.uniprot.org/uniprot/CE164_HUMAN CE164_HUMAN] Senior-Loken syndrome. The disease is caused by variants affecting the gene represented in this entry.
== Function ==
[https://www.uniprot.org/uniprot/CE164_HUMAN CE164_HUMAN] Plays a role in microtubule organization and/or maintenance for the formation of primary cilia (PC), a microtubule-based structure that protrudes from the surface of epithelial cells. Plays a critical role in G2/M checkpoint and nuclear divisions. A key player in the DNA damage-activated ATR/ATM signaling cascade since it is required for the proper phosphorylation of H2AX, RPA, CHEK2 and CHEK1. Plays a critical role in chromosome segregation, acting as a mediator required for the maintenance of genomic stability through modulation of MDC1, RPA and CHEK1.<ref>PMID:17954613</ref> <ref>PMID:18283122</ref> <ref>PMID:23348840</ref> 
<div style="background-color:#fffaf0;">
== Publication Abstract from PubMed ==
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.

Molecular mechanisms underlying the role of the centriolar CEP164-TTBK2 complex in ciliopathies.,Rosa E Silva I, Bino L, Johnson CM, Rutherford TJ, Neuhaus D, Andreeva A, Cajanek L, van Breugel M Structure. 2021 Sep 7. pii: S0969-2126(21)00302-6. doi:, 10.1016/j.str.2021.08.007. PMID:34499853<ref>PMID:34499853</ref>

From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
</div>
<div class="pdbe-citations 7o06" style="background-color:#fffaf0;"></div>
== References ==
<references/>
__TOC__
</StructureSection>
[[Category: Camelidae mixed library]]
[[Category: Homo sapiens]]
[[Category: Large Structures]]
[[Category: E Silva IR]]
[[Category: Van Breugel M]]