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The surfactant lipid transporter ABCA3 is N-terminally cleaved inside LAMP3-positive vesicles |
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| Authors: | Stefanie Engelbrecht Matthias Griese |
| Institution: | Pediatric Pneumology, Dr. von Hauner Children’s Hospital, Ludwig-Maximilians University, D-80337 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.Structured summaryMINT-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) |
| Keywords: | ABC transporter ATP-binding cassette transporter LB lamellar bodies MVB multivesicular bodies LAMP3 lysosomal-associated membrane protein 3 ILD interstitial lung disease ER endoplasmic reticulum YFP/GFP yellow/green fluorescent protein HA-tag hemagglutinin tag SP surfactant protein E-64 l-leucylamido-(4-guanidino)butane" target="_blank">trans-epoxysuccinyl-l-leucylamido-(4-guanidino)butane |
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