6A70

Structure of the human PKD1/PKD2 complex

6A70 の概要

• エントリーDOI: 10.2210/pdb6a70/pdb
• EMDBエントリー: 6991 6992
• 分子名称: Polycystin-2, Polycystin-1 (2 entities in total)
• 機能のキーワード: asymmetric complex, polycystic kidney disease, membrane protein
• 由来する生物種: Homo sapiens (Human); 詳細
• タンパク質・核酸の鎖数: 4
• 化学式量合計: 326895.05

構造登録者

Su, Q.,Hu, F.,Ge, X.,Lei, J.,Yu, S.,Wang, T.,Zhou, Q.,Mei, C.,Shi, Y. (登録日: 2018-06-29, 公開日: 2018-08-15, 最終更新日: 2024-03-27)

主引用文献

Su, Q.,Hu, F.,Ge, X.,Lei, J.,Yu, S.,Wang, T.,Zhou, Q.,Mei, C.,Shi, Y.
Structure of the human PKD1-PKD2 complex.
Science, 361:-, 2018

PubMed Abstract: Mutations in two genes, and , account for most cases of autosomal dominant polycystic kidney disease, one of the most common monogenetic disorders. Here we report the 3.6-angstrom cryo-electron microscopy structure of truncated human PKD1-PKD2 complex assembled in a 1:3 ratio. PKD1 contains a voltage-gated ion channel (VGIC) fold that interacts with PKD2 to form the domain-swapped, yet noncanonical, transient receptor potential (TRP) channel architecture. The S6 helix in PKD1 is broken in the middle, with the extracellular half, S6a, resembling pore helix 1 in a typical TRP channel. Three positively charged, cavity-facing residues on S6b may block cation permeation. In addition to the VGIC, a five-transmembrane helix domain and a cytosolic PLAT domain were resolved in PKD1. The PKD1-PKD2 complex structure establishes a framework for dissecting the function and disease mechanisms of the PKD proteins.

PubMed: 30093605
DOI: 10.1126/science.aat9819

実験手法

ELECTRON MICROSCOPY (3.6 Å)