Structure of the human PKD1/PKD2 complex

Mutations in two genes, pkd1 and pkd2, account for most cases of autosomal dominant polycystic kidney disease (ADPKD), one of the most common monogenetic disorders. Here we report the 3.6 Å cryo-EM 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 non-canonical, TRP channel architecture. The S6 helix in PKD1 is broken in the middle, with the…
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