Here, using PCK rats as a model, we showed that both ivermectin (P2X4R allosteric modulator) (Priel & Silberberg, 2004) and 5‐BDBD (P2X4R antagonist) (Coddou et al., 2019) did not affect the progression of ARPKD in PCK rats, which may provide insights for future investigation of the therapeutic potential of ATP‐P2 signaling in PKD diseases. The gene discussed is P2RX4; the disease is autosomal recessive polycystic kidney disease.