Article
Structure of the human PKD1-PKD2 complex.
Science (New York, N.Y.) - 7 Sept 2018
Su Qiang, Hu Feizhuo, Ge Xiaofei, Lei Jianlin, Yu Shengqiang, Wang Tingliang, Zhou Qiang, Mei Changlin, Shi Yigong
Abstract excerpt
Mutations in two genes, PKD1 and PKD2, 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,...
Topics
- Cryoelectron Microscopy
- Crystallography, X-Ray
- Humans
- Multiprotein Complexes
- Mutation
- Polycystic Kidney, Autosomal Dominant
- Protein Domains
- Protein Folding
- TRPP Cation Channels