The serum levels of HMGB1 are increased in ADPKD patients (Nakamura et al., 2011; Nakamura et al., 2012), while S100A8 and A9 were found to be remarkably increased in both ADPKD patients and mouse models (Lee et al., 2015). Here, HMGB1 is linked to autosomal dominant polycystic kidney disease.