The surfactant lipid transporter ABCA3 is N-terminally cleaved inside LAMP3-positive vesicles

被引：29

作者：

Engelbrecht, Stefanie ^{[1
]}

Kaltenborn, Eva ^{[1
]}

Griese, Matthias ^{[1
]}

Kern, Suncana ^{[1
]}

机构：

[1] Univ Munich, Dr von Hauner Childrens Hosp, D-80337 Munich, Germany

来源：

FEBS LETTERS | 2010年 / 584卷 / 20期

关键词：

ABCA3; ATP-binding cassette transporter; Surfactant; Alveolar epithelial type II cell; Lamellar body; Protease; INTERSTITIAL LUNG-DISEASE; PULMONARY SURFACTANT; CATHEPSIN-H; PROTEIN-B; INTRACELLULAR VESICLES; MEMBRANE-PROTEIN; SIGNAL PEPTIDES; II PNEUMOCYTES; CULTURED-CELLS; NAPSIN-A;

D O I：

10.1016/j.febslet.2010.09.026

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

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. Structured summary: MINT-7996633: Calnexin (uniprotkb:P27824) and ABCA3 (uniprotkb:Q99758) colocalize (MI:0403) by fluorescence microscopy (MI: 0416) MINT-7996380, MINT-7996593, MINT-7996607: LAMP3 (uniprotkb:Q9UQV4) and ABCA3 (uniprotkb: Q99758) colocalize (MI:0403) by fluorescence microscopy (MI:0416) (C) 2010 Federation of European Biochemical Societies. Published by Elsevier B.V. All rights reserved.

引用

页码：4306 / 4312

页数：7

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