Insights into pathobiology of ADPKD from human and animal genetic studies. AMPK, 5ʹ-AMP–activated protein kinase; ER, endoplasmic reticulum; ERK, extracellular signal–regulated kinase; JAK-STAT, Janus kinase-signal transducer and activator of transcription signaling pathway; mTOR, mammalian target of rapamycin.