Fig 1: PYCR1 deficiency regulates the mitochondrial structure and cellular metabolism of the airway epithelium and AKT/mTORC1 and Wnt3a/β-catenin signaling ex vivo.(A) The mitochondrial morphology alterations in primary tracheal epithelial cells from the 4 groups were observed by transmission electron microscopy (n = 3/group); original magnification 12,000×; scale bar, 1 μm. (B) The mitochondrial morphology alterations in alveolar epithelial cells from the 4 groups were observed by transmission electron microscopy; original magnification 12,000×; scale bar, 1 μm. (C) Representative ECAR profiles, changes in basal ECAR, and glycolytic capacity in primary airway epithelial cells from 4 groups. (D) Western blot analysis of p-AKT expression in lung tissues (n = 3/group). (E) Western blot analysis of p-Raptor expression in lung tissues (n = 3/group). (F) Western blot analysis of Wnt3a expression in lung tissues (n = 6/group). (G) Western blot analysis of β-catenin expression in lung tissues (n = 6/group). Bar graphs and data are presented as the means ± SEMs. Statistical analysis was performed using 1-way ANOVA followed by Bonferroni’s post hoc test. *P < 0.05, **P < 0.01, ***P < 0.001, ****P < 0.0001.