The canalicular multispecific organic anion transporter and conjugated hyperbilirubinemia in rat and man

J Mol Med (Berl). 1997 Jun;75(6):420-8. doi: 10.1007/s001090050127.


The human Dubin-Johnson syndrome is an autosomal recessive liver disease characterized by a chronic conjugated hyperbilirubinemia. Patients have impaired hepatobiliary transport of many endogenous and xenobiotic compounds. A similar disease phenotype has been described for a naturally occurring mutant Wistar rat strain, the TR- rat, which is defective in the, functionally defined, canalicular multispecific organic anion transporter (cMOAT). The complementary DNA encoding this protein has been cloned from rat and recently from human liver. cMOAT is a new member of the ATP-binding cassette transporter family, and homologous to the multidrug resistance-associated protein 1. A mutation in the cMOAT gene is responsible for the phenotype observed in TR- rats. This information should soon lead tc a complete genetic characterization of the human Dubin-Johnson syndrome.

Publication types

  • Review

MeSH terms

  • Animals
  • Anion Transport Proteins
  • Bile Acids and Salts / metabolism*
  • Bile Canaliculi / metabolism*
  • Carrier Proteins / genetics
  • Carrier Proteins / metabolism*
  • DNA, Complementary / isolation & purification
  • Humans
  • Hydroxysteroid Dehydrogenases*
  • Hyperbilirubinemia / metabolism*
  • Jaundice, Chronic Idiopathic / metabolism*
  • Membrane Glycoproteins*
  • Rats


  • Anion Transport Proteins
  • Bile Acids and Salts
  • Carrier Proteins
  • DNA, Complementary
  • Membrane Glycoproteins
  • bile acid binding proteins
  • Hydroxysteroid Dehydrogenases
  • AKR1C2 protein, human