Transporters involved in primary bile formation
| Transporter trivial name(s) | Gene code | Substrate(s) | Defective in |
|---|---|---|---|
| FIC1 | ATP8B1 | ? | PFIC type 1 |
| BSEP (sPgp) | ABCB11 | bile salts | PFIC type 2 |
| MDR3 Pgp (in mouse: Mdr2 Pgp) | ABCB4 | phospatidylcholine | PFIC 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 |