Decreased OLA1 expression contributes to SOD2 deficiency in PPHN. (A) Dual immunofluorescence staining for αSMA (green), OLA1 and DAPI nuclear staining showing decreased OLA1 expression in distal-PAs from PPHN patients compared to infants without PPHN (n=4). (B) Immunoblottings showing decreased OLA1 expression in the lungs, PAs, isolated PAECs, and PASMCs from PPHN compared to control-lambs (n=7). (C) Western blots and bar charts showing decreased OLA1 expression and SOD2 activity OLA1-siRNA treated PAECs and controls, with or without acetylcholine treatments (n=5). (D) Bar charts showing increased O2¯-specific oxidized mitoSOX product, 2-OH-Mito-E+ formation in OLA1-silenced PAECs, scrambled-siRNA, and mito-TEMPO-treated OLA1 deficient PAECs (n=5). (E) Bar charts showing the effects of OLA1-siRNA, scrambled-siRNA, and Lenti-OLA1 on the SOD2 gene promoter activity in normal PAECs (n=4). *p<.05