Bile salts play a crucial role in the digestion and absorption of dietary fats in the small intestine, particularly in the duodenum. The human bile salt export pump (BSEP), also known as ABCB11, mediates a variety of bile salts transport into canaliculi against a steep concentration gradient in the enterohepatic circulation. BSEP dysfunction, caused by mutations or induced by drugs, is frequently associated with severe cholestatic liver disease.