Larsen et al. (2018) showed that megalin is the antigen target of human kidney anti-brush border antibody that causes a primary renal tubulointerstitial disease (ABBA disease). Furthermore, megalin has been proposed to be involved in Fanconi’s syndrome and Dent’s syndrome, correlated with the increase in the pro-inflammatory response and TII (Piwon et al., 2000; Christensen et al., 2003; Batuman, 2007; Nakhoul and Batuman, 2011; Vicinanza et al., 2011; Gaide Chevronnay et al., 2014; Eshbach and Weisz, 2017; Wang et al., 2017; Cez et al., 2018). Here, LRP2 is linked to Fanconi renotubular syndrome.