TAILIEUCHUNG - Báo cáo y học: " Some ABCA3 mutations elevate ER stress and initiate apoptosis of lung epithelial cells"

Tuyển tập các báo cáo nghiên cứu về y học được đăng trên tạp chí y học 'Respiratory Research cung cấp cho các bạn kiến thức về ngành y đề tài:" Some ABCA3 mutations elevate ER stress and initiate apoptosis of lung epithelial cells. | Weichert et al. Respiratory Research 2011 12 4 http content 12 1 4 RESPIRATORY RESEARCH RESEARCH Open Access Some ABCA3 mutations elevate ER stress and initiate apoptosis of lung epithelial cells 11 11 1 1 1 2 Nina Weichert Eva Kaltenborn Andreas Hector Markus Woischnik Andrea Schams Andreas Holzinger Suncana Kern 1 Matthias Griese1 Abstract Background ABCA3 transporter ATP-binding cassette transporter of the A subfamily is localized to the limiting membrane of lamellar bodies organelles for assembly and storage of pulmonary surfactant in alveolar epithelial type II cells AECII . It transports surfactant phospholipids into lamellar bodies and absence of ABCA3 function disrupts lamellar body biogenesis. Mutations of the ABCA3 gene lead to fatal neonatal surfactant deficiency and chronic interstitial lung disease ILD of children. ABCA3 mutations can result in either functional defects of the correctly localized ABCA3 or trafficking folding defects where mutated ABCA3 remains in the endoplasmic reticulum ER . Methods Human alveolar epithelial A549 cells were transfected with vectors expressing wild-type ABCA3 or one of the three ABCA3 mutant forms R43L R280C and L101P C-terminally tagged with YFP or hemagglutinin-tag. Localization trafficking properties were analyzed by immunofluorescence and ABCA3 deglycosylation. Uptake of fluorescent NBD-labeled lipids into lamellar bodies was used as a functional assay. ER stress and apoptotic signaling were examined through RT-PCR based analyses of XBP1 splicing immunoblotting or FACS analyses of stress apoptosis proteins Annexin V surface staining and determination of the intracellular glutathion level. Results We demonstrate that two ABCA3 mutations which affect ABCA3 protein trafficking folding and lead to partial R280C or complete L101P retention of ABCA3 in the ER compartment can elevate ER stress and susceptibility to it and induce apoptotic markers in the cultured lung epithelial A549 cells. R43L .

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