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Updated: Jun 8, 2026

ABCG5/G8 Crystallization in a Lipidic Bicelle Environment for X-Ray Crystallography
Published on: August 25, 2023
The surfactant lipid transporter ABCA3 is N-terminally cleaved inside LAMP3-positive vesicles
Stefanie Engelbrecht1, Eva Kaltenborn, Matthias Griese
1Pediatric Pneumology, Dr. von Hauner Children's Hospital, Ludwig-Maximilians University, Munich, Germany.
Abstract:
ABCA3 mutations cause fatal surfactant deficiency and interstitial lung disease. ABCA3 protein is a lipid transporter indispensible for surfactant biogenesis and storage in lamellar bodies (LB). The protein folds in endoplasmic reticulum and is glycosylated in Golgi en route to the membrane of mature LB and their precursor multivesicular bodies (MVB). In immunoblots, C-terminally labeled ABCA3 appears as two protein bands of 150 and 190 kDa. Using N- and C-terminal protein tags and hindering ABCA3 processing we show that the 150 kDa protein represents the mature ABCA3 whose N-terminus is cleaved by a cysteine protease inside MVB/LB.
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