2016 JULY-1

The literature watch of this month focuses on the Rotor syndrome associated with OATP deficiency. Three articles discuss liver imaging. Illustration shows human liver biopsies with and without OATP.


  • Xenobiotica. 2016;46:457-66. Organic anion transporting polypeptide (OATP)-mediated transport of coproporphyrins I and III. Bednarczyk D, Boiselle C. Identification of coproporphyrins as OATP substrates in vitro more clearly defines the role of OATPs in the hepatic disposition and renal excretion of coproporphyrins I and III and provides compelling evidence for future in vivo exploration of coproporphyrins as biomarkers of OATP activity. PubMed
  • Pediatr Res. 2016;79:378-86. Inherited disorders of bilirubin clearance. Memon N, Weinberger BI, Hegyi T, Aleksunes LM. PubMed
  • Hum Mutat. 2015 Mar;36:327-32. Recessive inheritance of population-specific intronic LINE-1 insertion causes a rotor syndrome phenotype. Kagawa T, Oka A, Kobayashi Y, Hiasa Y, Kitamura T, Sakugawa H, Adachi Y, Anzai K, Tsuruya K, Arase Y, Hirose S, Shiraishi K, Shiina T, Sato T, Wang T, Tanaka M, Hayashi H, Kawabe N, Robinson PN, Zemojtel T, Mine T. PubMed
  • Clin Rev Allergy Immunol. 2015;48:243-53. Gene replacement therapy for genetic hepatocellular jaundice. van Dijk R1, Beuers U, Bosma PJ. Review of clinical features, pathophysiology and genetic background associated with inherited disorders of bilirubin metabolism and transport. PubMed
  • Pediatr Int. 2015;57:981-5. IMAGING. Radionuclide cholescintigraphy in genetically confirmed Rotor syndrome. Sirucek P, Sulakova A, Jirsa M, Mrhac L, Havel M, Kraft O. PubMed
  • Gastroenterology. 2014;146:1625-38.  Inherited disorders of bilirubin transport and conjugation: new insights into molecular mechanisms and consequences. Erlinger S, Arias IM, Dhumeaux D. PubMed
  • Oncol Lett. 2014;7:797-798.  Management of a patient with colon cancer and rotor syndrome: A case report. Arslan D, Avci F, Merdin A, Gunduz S, Coskun HS. PubMed
  • Drug Metab Dispos. 2014;42:561-5. The roles of MRP2, MRP3, OATP1B1, and OATP1B3 in conjugated hyperbilirubinemia. Keppler D. PubMed
  • World J Gastroenterol. 2013;19:6398-407. New insights in bilirubin metabolism and their clinical implications. Sticova E, Jirsa M. PubMed
  • J Hepatol. 2013;58:388-90. Hereditary conjugated hyperbilirubinaemia: 37 years later. Dhumeaux D, Erlinger S. PubMed
  • Cancer Biol Ther. 2012;13:1374-5. Loss of OATP1B3 function causes Rotor syndrome: implications for potential use of inhibitors in cancer. Pratt E, Sissung TM, Figg WD. PubMed
  • J Clin Invest. 2012;122:519-28. Complete OATP1B1 and OATP1B3 deficiency causes human Rotor syndrome by interrupting conjugated bilirubin reuptake into the liver. van de Steeg E, Stránecký V, Hartmannová H, Nosková L, Hřebíček M, Wagenaar E, van Esch A, de Waart DR, Oude Elferink RP, Kenworthy KE, Sticová E, al-Edreesi M, Knisely AS, Kmoch S, Jirsa M, Schinkel AH. PubMed
  • Best Pract Res Clin Gastroenterol. 2010;24:555-71. Hyperbilirubinemia syndromes (Gilbert-Meulengracht, Crigler-Najjar, Dubin-Johnson, and Rotor syndrome). Strassburg CP. PubMed
  • J Nucl Med. 1992;33:1550-1. IMAGING. Scintigraphic aspect of Rotor's disease with technetium-99m-mebrofenin. LeBouthillier G, Morais J, Picard M, Picard D, Chartrand R, Pomier G. PubMed
  • Radiology. 1982;142:743-6. IMAGING. 99mTc-HIDA cholescintigraphy in Dubin-Johnson and Rotor syndromes. Bar-Meir S, Baron J, Seligson U, Gottesfeld F, Levy R, Gilat T. PubMed


With an anti-OATP1B1/3 antibody, basolateral membrane immunostaining of hepatocytes in centrilobular areas is intense in control. Asterisks indicate central veins, arrowheads bile canaliculi, and crosses sinusoids. OATP1B proteins are not detectable in subjects with Rotor syndrome (CE1, CE3, and P1 II.1). PubMed