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Schematic representation of the major proteins involved in genetic cholestasis. BSEP is the bile acid (BA) transporter. MRP2 transports organic anion conjugates (OA), including bilirubin. MDR3 flops phosphatidylcholine (PC). FIC1 flips aminophospholipids (AL). The canalicular space is created by the apical membranes of adjacent hepatocytes and is contained by tight junction complexes; representative members of which are shown.
