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TitleMolecular dysregulation of ciliary polycystin-2 channels caused by variants in the TOP domain.
Journal, issue, pagesProc Natl Acad Sci U S A, Vol. 117, Issue 19, Page 10329-10338, Year 2020
Publish dateMay 12, 2020
AuthorsThuy N Vien / Jinliang Wang / Leo C T Ng / Erhu Cao / Paul G DeCaen /
PubMed AbstractGenetic variants in which encodes for the polycystin-2 ion channel are responsible for many clinical cases of autosomal dominant polycystic kidney disease (ADPKD). Despite our strong understanding ...Genetic variants in which encodes for the polycystin-2 ion channel are responsible for many clinical cases of autosomal dominant polycystic kidney disease (ADPKD). Despite our strong understanding of the genetic basis of ADPKD, we do not know how most variants impact channel function. Polycystin-2 is found in organelle membranes, including the primary cilium-an antennae-like structure on the luminal side of the collecting duct. In this study, we focus on the structural and mechanistic regulation of polycystin-2 by its TOP domain-a site with unknown function that is commonly altered by missense variants. We use direct cilia electrophysiology, cryogenic electron microscopy, and superresolution imaging to determine that variants of the TOP domain finger 1 motif destabilizes the channel structure and impairs channel opening without altering cilia localization and channel assembly. Our findings support the channelopathy classification of variants associated with ADPKD, where polycystin-2 channel dysregulation in the primary cilia may contribute to cystogenesis.
External linksProc Natl Acad Sci U S A / PubMed:32332171 / PubMed Central
MethodsEM (single particle)
Resolution3.24 Å
Structure data

21586

6wb8

EMDB-21586, PDB-6wb8:
Cryo-EM structure of PKD2 C331S disease variant
Method: EM (single particle) / Resolution: 3.24 Å

Chemicals

ChemComp-NAG:
2-acetamido-2-deoxy-beta-D-glucopyranose

Source
  • homo sapiens (human)
KeywordsTRANSPORT PROTEIN / PKD2

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