From: ATP-binding cassette (ABC) transporters in normal and pathological lung
ABC gene | Gene location | Functional role or substrates | Linked to disease | Protein expression in lung cells (+ localisation $ ) | References # |
---|---|---|---|---|---|
MDR1/P-gp (ABCB1) | 7q21.12 | Drug resistance, Hydrophobic organic cations | Unknown | Bronchial epithelium (ap), mucinous glands (ap), alveolar type I cells* (ap), endothelium* (ap), alv. macroph. | [4,12,14-16] |
MDR3/P-gp (ABCB4) | 7q21.12 | Phosphatidyl choline | Unknown | Absent | [4] |
MRP1 (ABCC1) | 16p13.12 | Drug resistance, Organic anions (e.g. GSH conjugates, LTC4) | COPD? | Bronchial epithelium (ba/la), goblet cells (ba), peripheral epithelial cells* (ba/la), seromucinous glands (ba/la), alv. macroph. | [4,53,85] |
MRP2 (ABCC2) | 10q24.2 | Drug resistance, Organic anions | Dubin-Johnson syndrome | Bronchial epithelium* (ap), primary bronchial and peripheral epithelial cells* (in) | [4,85,90] |
MRP3 (ABCC3) | 17q21.33 | Drug resistance, Organic anions | Unknown | Primary bronchial and peripheral epithelial cells* (ba/la) | [85] |
MRP4 (ABCC4) | 13q32.1 | Nucleoside analogues, Prostaglandin E1, E2 | Unknown | Primary bronchial and peripheral epithelial cells* (in) | [85] |
MRP5 (ABCC5) | 3q27.1 | Nucleoside analogues, Hyaluron | Unknown | Primary bronchial and peripheral epithelial cells* (in) | [85] |
MRP6 (ABCC6) | 16p13.12 | Unknown | Pseudoxanthoma elasticum | Unknown | Â |
MRP7 (ABCC10) | 6p21.1 | Drug resistance | Unknown | Unknown | Â |
MRP8 (ABCC11) | 16q12.1 | Conjugated steroids, Nucleoside analogues, bile acids | Unknown | Unknown | Â |
MRP9 (ABCC12) | 16q12.1 | Unknown | Unknown | Unknown | Â |
CFTR (ABCC7) | 7q31.31 | Chloride ion channel | Cystic fibrosis | Bronchial epithelium (ap), seromucinous glands (ap), Clara cells*, Alv. type I cells* | [117-120] |
BCRP (ABCG2) | 4q22 | Drug resistance, Protection food toxins | Unknown | Bronchial epithelium (ba/la), endothelium, seromucinous glands | [4] |
ABCA1 | 9q31.1 | Cholesterol and phospholipids | Tangier disease | Alv. type II cells | [151] |
ABCA3 | 16p13.3 | Surfactant secretion | Surfactant deficiency | Alv. type II cells | [155] |