Reactome | ABCA3 transports PC, PG from ER membrane to lamellar body

ABCA3 transports PC, PG from ER membrane to lamellar body

Stable Identifier

R-HSA-5683714

Type

Reaction [transition]

Species

Homo sapiens

Compartment

lamellar body membrane, lamellar body, cytosol, endoplasmic reticulum membrane

ReviewStatus

5/5

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ABCA3 transports PC, PG from ER membrane to lamellar body

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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