Figure 8
From: A large kindred of pulmonary fibrosis associated with a novel ABCA3 gene variant
Cellular effects of 48 h transient ABCA3 expression in A549 cells. a. ABCA3 mRNA expression levels analyzed by quantitative real time PCR (P < 0.001 for WT/G964D vs. A549/Mock). b. Western immunoblot analysis of HA-tagged ABCA3 in total cell lysates. β-actin was used as a loading control. c. Ratio of the upper/lower ABCA3 processing form. d. Co-immunostaining of HA-tagged ABCA3 with the lysosomal (lamellar body) marker LAMP3. e. Co-staining of HA-tagged ABCA3 with the ER marker calnexin. f. ER (calnexin, BiP) and early endosome markers (EEA1) in cells transiently transfected with ABCA3. β-actin was used as a loading control. Scale bars 7.5 μm, *P < 0.05.