Fig. 1

HPS-2 alters mitochondrial structure and function in the alveolar epithelium of the lung. a Representative TEM images of AE2 cells from lungs of WT and HPS-2 mice. Mitochondria from HPS-2 mice show smaller mitochondria (red arrow) with reduced number of cristae. Bar graph depicts number of abnormally-shaped mitochondria per AE2 cell (n = 15 per group). b, c Mitochondrial Complex I and IV activity in mitochondrial isolates from lungs of WT and HPS-2 mice (n = 6, each group). d Levels of ETC protein subunits in lungs of WT and HPS-2 mice. e Densitometry analysis of ETC protein complexes. f Mitochondrial ROS levels in alveolar epithelial (AE2) cells from lungs of WT and HPS-2 mice (n = 5, each group). g, h Aconitase and fumarase activity in AE2 cells from lungs of WT and HPS-2 mice (n = 6, each group). Statistical significance was assessed by Student’s t-test. *P < 0.05, **P < 0.01 vs. control group. Densitometry analyses (bar graphs) are representative n = 5 or more mouse specimens