ABCA3 transports PC, PG from ER membrane to lamellar body

Stable Identifier
Reaction [transition]
Homo sapiens
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ATP-binding cassette sub-family A member 3 (ABCA3) plays an important role in the formation of pulmonary surfactant, probably by transporting phospholipids such as phosphatidylcholine (PC) and phosphatidylglycerol (PG) from the ER membrane to lamellar bodies (LBs). PC and PG are the major phospholipid constituents of pulmonary surfactant. LBs are the surfactant storage organelles of type II epithelial cells from where phospholipids can be secreted together with surfactant proteins (SFTPs) into the alveolar airspace (Klugbauer & Hofmann 1996, Yamano et al. 2001). Defects in ABCA3 are the cause of pulmonary surfactant metabolism dysfunction type 3 (SMDP3; MIM:610921) (Shulenin et al. 2004).

Literature References
PubMed ID Title Journal Year
8706931 Primary structure of a novel ABC transporter with a chromosomal localization on the band encoding the multidrug resistance-associated protein

Klugbauer, N, Hofmann, F

FEBS Lett. 1996
15044640 ABCA3 gene mutations in newborns with fatal surfactant deficiency

Wert, SE, Annilo, T, Whitsett, JA, Nogee, LM, Shulenin, S, Dean, M

N Engl J Med 2004
11718719 ABCA3 is a lamellar body membrane protein in human lung alveolar type II cells

Morohoshi, T, Ban, N, Kawanami, O, Inagaki, N, Yamano, G, Shioda, S, Uchida, Y, Zhao, LX, Funahashi, H, Ogawa, J

FEBS Lett 2001
Catalyst Activity

lipid transporter activity of ABCA3 [lamellar body membrane]

Orthologous Events
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