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. 2014 Nov 18;5:5482. doi: 10.1038/ncomms6482

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PC1 is cleaved at GPS and forms a complex with PC2 in the ER (1). Rabep1 binds PC1 cytoplasmic C-terminal tail at a pre-Golgi compartment (2), and this complex traffics to the trans-Golgi network (TGN). At the TGN, Rabep1 couples the polycystin complex to a GGA1/Arl3 module (3), which is formed by Arl3-GTP binding to GGA1 via the GAT domain. GGA1 then assembles the clathrin coat (4) to form the vesicle carrier (5). The resulting polycystin complex-bearing vesicle traffics along the cytoplasmic microtubules to the base of cilia (6) and enters the cilium by an unknown mechanism.