P2RX4 and autosomal recessive polycystic kidney disease: In conclusion, this study revealed that modulation of P2X4R signaling by ivermectin (P2X4R‐potentiating drug) or 5‐BDBD (P2X4R antagonist) does not affect the development of ARPKD in PCK rats, which may provide insights for future studies on investigating the therapeutic potential of adenosine triphosphate (ATP)‐P2 signaling in PKD diseases.