7O0S

Crystal structure of the N-terminal domain of CEP164(1-109) bound to camelid nanobody 36Z

7O0S の概要

• エントリーDOI: 10.2210/pdb7o0s/pdb
• 関連するPDBエントリー: 7NWJ 7O06
• 分子名称: Nanobody 36Z, Centrosomal protein of 164 kDa (3 entities in total)
• 機能のキーワード: centriole centrosome basal body ciliogenesis, structural protein
• 由来する生物種: Camelidae mixed library; 詳細
• タンパク質・核酸の鎖数: 2
• 化学式量合計: 26177.04

構造登録者

e Silva, I.R.,van Breugel, M. (登録日: 2021-03-26, 公開日: 2021-09-15, 最終更新日: 2024-01-31)

主引用文献

Rosa E Silva, I.,Bino, L.,Johnson, C.M.,Rutherford, T.J.,Neuhaus, D.,Andreeva, A.,Cajanek, L.,van Breugel, M.
Molecular mechanisms underlying the role of the centriolar CEP164-TTBK2 complex in ciliopathies.
Structure, 30:114-128.e9, 2022

PubMed Abstract: 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.

PubMed: 34499853
DOI: 10.1016/j.str.2021.08.007

実験手法

X-RAY DIFFRACTION (1.7 Å)