ABCA3 as a lipid transporter in pulmonary surfactant biogenesis.

The Journal of biological chemistry (2007-02-03)
Nobuhiro Ban, Yoshihiro Matsumura, Hiromichi Sakai, Yasukazu Takanezawa, Mayumi Sasaki, Hiroyuki Arai, Nobuya Inagaki
RESUMEN

ABCA3 protein is expressed predominantly at the limiting membrane of the lamellar bodies in alveolar type II cells, and mutations in the ABCA3 gene cause lethal respiratory distress in newborn infants. To investigate the function of ABCA3 protein, we generated Abca3-deficient mice by targeting Abca3. Full-term Abca3(-/-) newborn pups died within an hour after birth because of acute respiratory failure. Ultrastructural analysis revealed abnormally dense lamellar body-like organelles and no normal lamellar bodies in Abca3(-/-) alveolar type II cells. TLC and electrospray ionization mass spectrometry analyses of lipids in the pulmonary interstitium showed that phosphatidylcholine and phosphatidylglycerol, which contain palmitic acid and are abundant in normal surfactant lipids, were dramatically decreased in Abca3(-/-) lung. These findings indicate that ABCA3 plays an essential role in pulmonary surfactant lipid metabolism and lamellar body biogenesis, probably by transporting these lipids as substrates.

MATERIALES
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Avanti
14:0 PG, 1,2-dimyristoyl-sn-glycero-3-phospho-(1′-rac-glycerol) (sodium salt), powder
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14:1 (Δ9-Cis) PC, 1,2-dimyristoleoyl-sn-glycero-3-phosphocholine, chloroform
Sigma-Aldrich
Monoclonal Anti-β-Tubulin I+II antibody produced in mouse, clone JDR.3B8, ascites fluid
Sigma-Aldrich
Anti-ABCA3 antibody produced in rabbit, Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution

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