The Dubin–Johnson Syndrome is a rare hereditary disease, which is associated with hyperbilirubinemia (high bilirubin and bilirubin-glucuronide plasma concentrations).
- The disease is caused by loss of function mutations in the ABCC2 gene, which is coding for the ABC-transporter MRP2 (also termed as multispecific organic anion transporter (MOAT)).
- Normally, this transporter is localized in the canalicular membrane of hepatocytes, where it eliminates bilirubin-glucuronide and other organic anions into the bile.