Plays an important role in the formation of pulmonary surfactant, probably by transporting lipids such as cholesterol.
Highly expressed in lung, followed by brain, pancreas, skeletal muscle and heart. Weakly expressed in placenta, kidney and liver. Also expressed in medullary thyroid carcinoma cells (MTC) and in C-cell carcinoma.
Involvement in disease
Defects in ABCA3 are the cause of pulmonary surfactant metabolism dysfunction type 3 (SMDP3) [MIM:610921]; also called pulmonary alveolar proteinosis due to ABCA3 deficiency. A rare lung disorder due to impaired surfactant homeostasis. It is characterized by alveolar filling with floccular material that stains positive using the periodic acid-Schiff method and is derived from surfactant phospholipids and protein components. Excessive lipoproteins accumulation in the alveoli results in severe respiratory distress.
Belongs to the ABC transporter superfamily. ABCA family. Contains 2 ABC transporter domains.
Multifunctional polypeptide with two homologous halves, each containing an hydrophobic membrane-anchoring domain and an ATP binding cassette (ABC) domain.
ATP binding cassette sub family A (ABC1) member 3 antibody
ATP binding cassette sub family A member 3 antibody
ATP binding cassette transporter 3 antibody
ATP-binding cassette 3 antibody
ATP-binding cassette sub-family A member 3 antibody
ATP-binding cassette transporter 3 antibody
CED7. C. elegans, homolog of antibody
LBM 180 antibody
P180 Lamellar Body Protein antibody
Immunocytochemistry/ Immunofluorescence - Anti-ATP-binding cassette sub-family A member 3 antibody (ab99856)
ICC/IF image of ab99856 stained T47D cells. The cells were 4% formaldehyde fixed (10 min) and then incubated in 1%BSA / 10% normal goat serum / 0.3M glycine in 0.1% PBS-Tween for 1h to permeabilise the cells and block non-specific protein-protein interactions. The cells were then incubated with the antibody ab99856 at 1µg/ml overnight at +4°C. The secondary antibody (green) was DyLight® 488 goat anti- rabbit (ab96899) IgG (H+L) used at a 1/250 dilution for 1h. Alexa Fluor® 594 WGA was used to label plasma membranes (red) at a 1/200 dilution for 1h. DAPI was used to stain the cell nuclei (blue) at a concentration of 1.43µM.
Western blot - Anti-ATP-binding cassette sub-family A member 3 antibody (ab99856)
All lanes : Anti-ATP-binding cassette sub-family A member 3 antibody (ab99856) at 1/1000 dilution
Lane 1 : SW480 cell lysate Lane 2 : Hela cell lysate Lane 3 : Human fetal kidney lysate Lane 4 : Human fetal lung lysate