PT  - JOURNAL ARTICLE
AU  - Frans J. C. Cuperus
AU  - Thierry Claudel
AU  - Julien Gautherot
AU  - Emina Halilbasic
AU  - Michael Trauner
TI  - The Role of Canalicular ABC Transporters in Cholestasis
AID  - 10.1124/dmd.113.056358
DP  - 2014 Apr 01
TA  - Drug Metabolism and Disposition
PG  - 546--560
VI  - 42
IP  - 4
4099  - http://dmd.aspetjournals.org/content/42/4/546.short
4100  - http://dmd.aspetjournals.org/content/42/4/546.full
SO  - Drug Metab Dispos2014 Apr 01; 42
AB  - Cholestasis, a hallmark feature of hepatobiliary disease, is characterized by the retention of biliary constituents. Some of these constituents, such as bile acids, inflict damage to hepatocytes and bile duct cells. This damage may lead to inflammation, fibrosis, cirrhosis, and eventually carcinogenesis, sequelae that aggravate the underlying disease and deteriorate clinical outcome. Canalicular ATP-binding cassette (ABC) transporters, which mediate the excretion of individual bile constituents, play a key role in bile formation and cholestasis. The study of these transporters and their regulatory nuclear receptors has revolutionized our understanding of cholestatic disease. This knowledge has served as a template to develop novel treatment strategies, some of which are currently already undergoing phase III clinical trials. In this review we aim to provide an overview of the structure, function, and regulation of canalicular ABC transporters. In addition, we will focus on the role of these transporters in the pathogenesis and treatment of cholestatic bile duct and liver diseases.