Multidrug resistance-associated protein 6 (IPR030239)
Short name: ABCC6
Overlapping homologous superfamilies
- Multi drug resistance-associated protein (IPR005292)
- Multidrug resistance-associated protein 6 (IPR030239)
This entry represents the multidrug resistance-associated protein 6 (MRP6 or ABCC6) from animals. It belongs to the MRP (multidrug resistance protein) subfamily of the ATP-binding cassette (ABC) transporter family [PMID: 21103974]. It is a multispecific organic anion transporter able to export glutathione conjugates including lencotriene C4, DNP, and N-ethylmaleimide S-glutathione; it also exports anthracyclines, epipodophyllotoxins, cisplatin, and probably exports probenecid, benzbromarone and indomethacin [PMID: 21740521].
ABCC6 deficiency causes the ectopic mineralisation disorder pseudoxanthoma elasticum (PXE), characterised by calcification and fragmentation of elastic fibres, resulting in oculocutaneous and cardiovascular symptoms [PMID: 24137173].
The ABC transporter family is a group of membrane proteins that use the hydrolysis of ATP to power the translocation of a wide variety of substrates across cellular membranes. ABC transporters minimally consist of two conserved regions: a highly conserved nucleotide-binding domain (NBD) and a less conserved transmembrane domain (TMD). Eukaryotic ABC proteins are usually organised either as full transporters (containing two NBDs and two TMDs), or as half transporters (containing one NBD and one TMD), that have to form homo- or heterodimers in order to constitute a functional protein [PMID: 11441126].
- PTHR24223:SF339 (PTHR24223:SF339)