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Structure of the human PKD1-PKD2 complex


ABSTRACT:

SUBMITTER: Yigong Shi 

PROVIDER: EMPIAR-10262 | biostudies-other |

REPOSITORIES: biostudies-other

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Structure of the human PKD1-PKD2 complex.

Su Qiang Q   Hu Feizhuo F   Ge Xiaofei X   Ge Xiaofei X   Lei Jianlin J   Yu Shengqiang S   Wang Tingliang T   Zhou Qiang Q   Mei Changlin C   Shi Yigong Y  

Science (New York, N.Y.) 20180809 6406


Mutations in two genes, <i>PKD1</i> and <i>PKD2</i>, 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 P  ...[more]

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