Figure 4

BTHS lymphoblasts have larger mitochondria that are reduced in size to healthy lymphoblast levels by OEA treatment. Representative whole cell and subcellular mitochondrial images are shown for vehicle-treated healthy lymphoblasts (a–c), 1 µM OEA-treated healthy lymphoblasts (d–f), vehicle-treated BTHS lymphoblasts (g–i), and 1 µM OEA-treated BTHS lymphoblasts (j–l). The white arrow in (g) shows a characteristic, atypically enlarged mitochondrion from a BTHS lymphoblast, while the image in (h) shows a mitochondrion with a ‘bubbly’ appearance, and the image in (i) shows a mitochondrion with an ‘onion-like’ appearance. Quantitative measures were derived from images produced from three of the BTHS and healthy lymphoblast lines after either vehicle treatment or treatment with 1 µM OEA for 4 days. The average number of mitochondria per cell (normalized to nuclear area to account for differences in cross-sectional area) is shown (m). Measures of mitochondrial morphology, including mitochondrial circularity (n), average total parallel cristae length per mitochondrion (o), average mitochondrial size (p), and the prevalence of atypically large mitochondria (q) are also reported. Scale bars = 500 nm. Data are means ± S.E.M; n = 3. #P = 0.06, *P < 0.05, **P < 0.01, ***P < 0.001.