Figure 2. Possible mechanisms involved in the occurrence of dRTA in patients with autoimmune liver disease. (A) Reduced AE2 expression (congenital or caused by proteolysis) could be related to changes in intracellular pH. This could lead to protein mistargeting in liver cells and in kidney tubules, antigenic changes, and autoimmunity. The immunoglobulin G autoantibodies produced destroy the chloride/bicarbonate exchanger (band 3 isoform) present in hepatocytes and in the basolateral membrane in alpha-type intercalated cells in the collecting tubule [12]. (B) Hepatocyte damage can initiate an immune response due to the cross-reaction between membrane proteins in hepatocytes and Tamm-Horsfall glycoprotein in renal tubular cells [15].