Phospholipid-transporting ATPase ABCA3

Definition:

Catalyzes the ATP-dependent transport of phospholipids such as phosphatidylcholine and phosphoglycerol from the cytoplasm into the lumen side of lamellar bodies, in turn participates in the lamellar bodies biogenesis and homeostasis of pulmonary surfactant. Transports preferentially phosphatidylcholine containing short acyl chains. In addition plays a role as an efflux transporter of miltefosine across macrophage membranes and free cholesterol (FC) through intralumenal vesicles by removing FC from the cell as a component of surfactant and protects cells from free cholesterol toxicity.

References:

[1]. Thomas Wittmann, et al. Tools to explore ABCA3 mutations causing interstitial lung disease. Pediatr Pulmonol. 2016 Dec;51(12):1284-1294. [Content Brief]

[2]. Ralf Zarbock, et al. ABCA3 protects alveolar epithelial cells against free cholesterol induced cell death. Biochim Biophys Acta. 2015 Jul;1851(7):987-95. [Content Brief]

[3]. Yoshihiro Matsumura, et al. ABCA3-mediated choline-phospholipids uptake into intracellular vesicles in A549 cells. FEBS Lett. 2007 Jul 10;581(17):3139-44. [Content Brief]

[4]. Yang Li, et al. Metabolic labelling of choline phospholipids probes ABCA3 transport in lamellar bodies. Biochim Biophys Acta Mol Cell Biol Lipids. 2019 Dec;1864(12):158516. [Content Brief]

[5]. Yoshihiro Matsumura, et al. Characterization and classification of ATP-binding cassette transporter ABCA3 mutants in fatal surfactant deficiency. J Biol Chem. 2006 Nov 10;281(45):34503-14. [Content Brief]

[6]. Stefanie Höppner, et al. Quantification of volume and lipid filling of intracellular vesicles carrying the ABCA3 transporter. Biochim Biophys Acta Mol Cell Res. 2017 Dec;1864(12):2330-2335. [Content Brief]

[7]. Luuk C T Dohmen, et al. Functional Validation of ABCA3 as a Miltefosine Transporter in Human Macrophages: IMPACT ON INTRACELLULAR SURVIVAL OF LEISHMANIA (VIANNIA) PANAMENSIS. J Biol Chem. 2016 Apr 29;291(18):9638-47. [Content Brief]

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