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multidrug resistance-associated protein 6; ATP-binding cassette sub-family C member 6; anthracycline resistance-associated protein; multi-specific organic anion transporter E; MOAT-E (ABCC6 ARA MRP6)
Function:
- may participate directly in the active transport of drugs into subcellular organelles or influence drug distribution indirectly
- transports glutathione conjugates including leukotriene-C4 (LTC4) & N-ethylmaleimide S-glutathione (NEM-GS)
Structure:
- belongs to the ABC transporter family, conjugate transporter (TC 3.A.1.208) subfamily
- contains 2 ABC transmembrane type-1 domains; residues 311-593 & 947-1228
- contains 2 ABC transporter domains; residues 629-853 & 1265-1499
Compartment: membrane
Expression:
- expressed in kidney & liver
- very low expression in other tissues
Pathology:
- defects in ABCC6 are the cause of pseudoxanthoma elasticum
General
ATP-binding cassette sub-family C (ABC transporter-C, ABCC)
glycoprotein
phosphoprotein
transmembrane 17 protein
Properties
SIZE: entity length = 1503 aa
MW = 165 kD
COMPARTMENT: cellular membrane
MOTIF: exoplasmic domain {1-31}
MOTIF: N-glycosylation site {N15}
transmembrane domain {32-52}
cytoplasmic loop {53-72}
transmembrane domain {73-93}
exoplasmic loop {94-98}
transmembrane domain {99-119}
cytoplasmic loop {120-131}
transmembrane domain {132-149}
exoplasmic loop {150-167}
transmembrane domain {168-188}
cytoplasmic loop {189-302}
MOTIF: Ser phosphorylation site {S244}
transmembrane domain {303-323}
exoplasmic loop {324-349}
transmembrane domain {350-370}
cytoplasmic loop {371-426}
transmembrane domain {427-447}
exoplasmic loop {448-450}
transmembrane domain {451-471}
cytoplasmic loop {472-533}
transmembrane domain {534-554}
exoplasmic loop {555-575}
transmembrane domain {576-596}
cytoplasmic loop {597-939}
MOTIF: ATP-binding site
NAME: ATP-binding site
SITE: 663-670
Ser phosphorylation site {S902}
transmembrane domain {940-960}
exoplasmic loop {961-997}
transmembrane domain {998-1018}
cytoplasmic loop {1019-1061}
transmembrane domain {1062-1082}
exoplasmic loop {1083-1083}
transmembrane domain {1084-1104}
cytoplasmic loop {1105-1175}
transmembrane domain {1176-1196}
exoplasmic loop {1197-1198}
transmembrane domain {1199-1219}
cytoplasmic domain {1220-1503}
MOTIF: ATP-binding site
NAME: ATP-binding site
SITE: 1299-1306
Database Correlations
OMIM correlations
MORBIDMAP 603234
UniProt O95255
PFAM correlations
Entrez Gene 368
References
- UniProt :accession O95255
- Mutations of the ABCC6 gene
Retina International's Scientific Newsletter
http://www.retina-international.com/sci-news/abcc6mut.htm
- GeneReviews
https://www.genecards.org/cgi-bin/carddisp.pl?gene=ABCC6