Table 1

Transporters involved in primary bile formation

Transporter trivial name(s)Gene codeSubstrate(s)Defective in
FIC1 ATP8B1 ?PFIC type 1
BSEP (sPgp) ABCB11 bile saltsPFIC type 2
MDR3 Pgp (in mouse: Mdr2 Pgp) ABCB4 phospatidylcholinePFIC type 3
MDR1 Pgp (in mouse: Mdr1a and 1b Pgp) ABCB1 amphipathic drugs (neutral and cationic)?
MRP2 (cMOAT) ABCC2 amphipathic drugs (anionic and neutral)Dubin Johnson syndrome
BCRP (MXR, ABCP) ABCG2 amphipathic drugs?
ABCG5 ABCG5 (phytosterols)Sitosterolaemia
ABCG8 ABCG8 no direct evidence yet