6XT9

Subunits BBS 1,4,8,9,18 of the human BBSome complex

6XT9 の概要

• エントリーDOI: 10.2210/pdb6xt9/pdb
• EMDBエントリー: 10617 10618
• 分子名称: Bardet-Biedl syndrome 1 protein, Bardet-Biedl syndrome 4 protein, Tetratricopeptide repeat domain 8 isoform 2, ... (5 entities in total)
• 機能のキーワード: ciliary transport, arl6 effector, adaptor protein, complex, protein transport
• 由来する生物種: Homo sapiens (Human); 詳細
• タンパク質・核酸の鎖数: 5
• 化学式量合計: 298139.56

構造登録者

Klink, B.U.,Raunser, S.,Gatsogiannis, C. (登録日: 2020-01-15, 公開日: 2020-01-29, 最終更新日: 2024-05-22)

主引用文献

Klink, B.U.,Gatsogiannis, C.,Hofnagel, O.,Wittinghofer, A.,Raunser, S.
Structure of the human BBSome core complex.
Elife, 9:-, 2020

PubMed Abstract: The BBSome is a heterooctameric protein complex that plays a central role in primary cilia homeostasis. Its malfunction causes the severe ciliopathy Bardet-Biedl syndrome (BBS). The complex acts as a cargo adapter that recognizes signaling proteins such as GPCRs and links them to the intraflagellar transport machinery. The underlying mechanism is poorly understood. Here we present a high-resolution cryo-EM structure of a human heterohexameric core subcomplex of the BBSome. The structure reveals the architecture of the complex in atomic detail. It explains how the subunits interact with each other and how disease-causing mutations hamper this interaction. The complex adopts a conformation that is open for binding to membrane-associated GTPase Arl6 and a large positively charged patch likely strengthens the interaction with the membrane. A prominent negatively charged cleft at the center of the complex is likely involved in binding of positively charged signaling sequences of cargo proteins.

PubMed: 31951201
DOI: 10.7554/eLife.53910

実験手法

ELECTRON MICROSCOPY (3.8 Å)