Interaction Proteomics of Polycystins 1 and 2 Reveal a Novel Role for the BLOC-1/BORC Lysosomal Positioning Complex.

ABSTRACT: PKD1 and PKD2 are the most commonly mutated genes in autosomal dominant polycystic kidney disease (ADPKD). However, the precise roles of the encoded polycystin 1/2 (PC1 and PC2) proteins, and how their functions are disrupted in ADPKD, remain unclear. Here, we characterize the protein interaction networks of PC1 and PC2 in cycling and ciliated cells using proximity-dependent biotinylation (BioID), identifying a common set of 172 proteins that interact with the C terminus of PC1 and the full-length PC2 protein, enriched in autophagy regulators, endoplasmic reticulum tethers, endoplasmic reticulum stress proteins, and other proteins previously linked to ADPKD. Notably, we also find that PC1 specifically interacts with ciliary and lysosomal proteins, including components of the biogenesis of

SUBMITTER: Lukmani F 

PROVIDER: S-EPMC12663635 | biostudies-literature | 2025 Oct

REPOSITORIES: biostudies-literature